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Journal articles on the topic 'MDX'

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Published: 4 June 2021
Last updated: 25 May 2024

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1

Banks, Glen B., Ariana C. Combs, and Jeffrey S. Chamberlain. "Sequencing protocols to genotype mdx , mdx 4cv , and mdx 5cv mice." Muscle & Nerve 42, no. 2 (May 18, 2010): 268–70. http://dx.doi.org/10.1002/mus.21700.

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2

Dempsey, Fiona C., Hussein Al-Ali, Scott J. Crichton, Charlene Fabian, Chris Pepper, Bin-Zhi Qian, Xue-Feng Li, and Christopher N. Parris. "Abstract 5294: MDX-124, a novel annexin-A1 antibody, induces significant anti-cancer activity in multiple preclinical models." Cancer Research 82, no. 12_Supplement (June 15, 2022): 5294. http://dx.doi.org/10.1158/1538-7445.am2022-5294.

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Abstract Annexin-A1 (ANXA1) is secreted from both cancer and immune cells in response to several physiological stimuli and modulates cellular functions through interactions with formyl peptide receptors (FPR1/2). Overexpression of ANXA1 has been observed in multiple cancers, including triple-negative breast (TNBC), colorectal, lung, pancreatic, gastric and prostate, and correlates with poor prognosis and decreased overall survival. ANXA1 has also been shown to promote cancer cell proliferation, angiogenesis, migration and drug resistance, and to modulate the tumor microenvironment. MDX-124 is a novel humanized antibody targeting ANXA1. Previously we presented data demonstrating its significant anti-proliferative activity. Here we provide further data on the mechanism of action of MDX-124, notably its impact on tumor growth, cell cycle arrest and migration in several preclinical cancer models. Incubation of pancreatic (BxPC-3), lung (A549) and TNBC (MDA-MB-231) cancer cell lines with MDX-124 for 24 h decreased the proportion of cells in S-phase by up to 18.3% with a concomitant increase in G1 phase of up to 33.5% versus untreated cells. This effect occurred in a dose-dependent manner and is consistent with an MDX-124 mediated increase in cell cycle arrest. After 72 h incubation with MDX-124, the migratory ability of gastric (AGS), prostate (PC-3), TNBC (MDA-MB-231), lung (A549), pancreatic (MIA PaCa-2) and colorectal (LoVo) cancer cells was significantly reduced in a dose-dependent manner when compared to untreated controls. Proteomic analysis following incubation of MDX-124 with a panel of cancer cell lines for 72 h demonstrated substantial alterations in the level of expression and phosphorylation of multiple key oncogenic proteins. In the MycCaP-Bo syngeneic model of bone metastatic prostate cancer, mice treated with the murine analog of MDX-124 (10 mg/kg, BIW) had a 52% reduction in mean tumor growth after 14 days compared to isotype control treated mice. In conclusion, our data indicate that targeting ANXA1 with MDX-124 inhibits key tumorigenic processes in several clinically challenging cancer indications. MDX-124 therefore provides an innovative approach to cancer therapy. Medannex initiated a First-In-Human study in Q4 2021 to evaluate MDX-124 in solid malignancies known to overexpress ANXA1. Citation Format: Fiona C. Dempsey, Hussein Al-Ali, Scott J. Crichton, Charlene Fabian, Chris Pepper, Bin-Zhi Qian, Xue-Feng Li, Christopher N. Parris. MDX-124, a novel annexin-A1 antibody, induces significant anti-cancer activity in multiple preclinical models [abstract]. In: Proceedings of the American Association for Cancer Research Annual Meeting 2022; 2022 Apr 8-13. Philadelphia (PA): AACR; Cancer Res 2022;82(12_Suppl):Abstract nr 5294.
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3

&NA;. "MDX RA." Drugs in R & D 3, no. 2 (2002): 111–12. http://dx.doi.org/10.2165/00126839-200203020-00008.

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4

Chechin, A. I., and C. D. Prokudaylo. "MDX-station." Nuclear Instruments and Methods in Physics Research Section A: Accelerators, Spectrometers, Detectors and Associated Equipment 448, no. 1-2 (June 2000): 120–21. http://dx.doi.org/10.1016/s0168-9002(99)00735-4.

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5

Heeps, Graham. "Acura MDX." Vehicle Dynamics International 2021, no. 1 (May 2021): 4–7. http://dx.doi.org/10.12968/s1479-7747(22)50142-7.

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6

Dubinin, Mikhail V., Irina B. Mikheeva, Anastasia E. Stepanova, Anastasia D. Igoshkina, Alena A. Cherepanova, Alena A. Semenova, Vyacheslav A. Sharapov, Igor I. Kireev, and Konstantin N. Belosludtsev. "Mitochondrial Transplantation Therapy Ameliorates Muscular Dystrophy in mdx Mouse Model." Biomolecules 14, no. 3 (March 7, 2024): 316. http://dx.doi.org/10.3390/biom14030316.

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Duchenne muscular dystrophy is caused by loss of the dystrophin protein. This pathology is accompanied by mitochondrial dysfunction contributing to muscle fiber instability. It is known that mitochondria-targeted in vivo therapy mitigates pathology and improves the quality of life of model animals. In the present work, we applied mitochondrial transplantation therapy (MTT) to correct the pathology in dystrophin-deficient mdx mice. Intramuscular injections of allogeneic mitochondria obtained from healthy animals into the hind limbs of mdx mice alleviated skeletal muscle injury, reduced calcium deposits in muscles and serum creatine kinase levels, and improved the grip strength of the hind limbs and motor activity of recipient mdx mice. We noted normalization of the mitochondrial ultrastructure and sarcoplasmic reticulum/mitochondria interactions in mdx muscles. At the same time, we revealed a decrease in the efficiency of oxidative phosphorylation in the skeletal muscle mitochondria of recipient mdx mice accompanied by a reduction in lipid peroxidation products (MDA products) and reduced calcium overloading. We found no effect of MTT on the expression of mitochondrial signature genes (Drp1, Mfn2, Ppargc1a, Pink1, Parkin) and on the level of mtDNA. Our results show that systemic MTT mitigates the development of destructive processes in the quadriceps muscle of mdx mice.
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7

Bowo, Prasetyo, Sumarmi Sumarmi, and Sri Hardiatmi. "PENERAPAN MACAM DAN DOSIS PUPUK ORGANIK TERHADAP PERTUMBUHAN DAN HASIL TANAMAN GANDUM (Triticum aestivum L.)." Innofarm:Jurnal Inovasi Pertanian 21, no. 1 (November 27, 2019): 1. http://dx.doi.org/10.33061/innofarm.v21i1.3311.

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Penelitian tentang “Penerapan macam dan dosis pupuk organik terhadap pertumbuhan dan hasil tanaman Gandum (Triticum aestivum L.)’’ telah dilaksanakan tanggal 08 November 2018 sampai 31 januari 2019 di Green House Fakultas Pertanian Universitas Slamet Riyadi Surakarta. Penelitian ini bertujuan untuk mengetahui dan menentukan pengaruh terbaik terhadap pertumbuhan dan hasil tanaman gandum. Rancangan penelitian yang digunakan adalah rancangan acak lengkap yang terdiri dari 7 perlakuan dan 5 ulangan. Perlakuan : 1) MD0 = tanpa pupk (kontrol), 2) MD1= pupuk kandang dosis 200 g/polybag, 3) MD2 = pupuk kandang dosis 400 g/polybag , 4) MD3 = pupuk guano dosis 200 g/polybag, 5) MD4 = pupuk guano dosis 400 g/polybag, 6) MD5 = pupuk kascing dosis 200 g/polybag, 7) MD6 = pupuk kascing dosis 400 g/polybag. Hasil penelitian menunjukkan bahwa : 1) penerapan macam dan dosis pupuk organik berpengaruh terhadap tinggi tanaman, jumlah daun, dan jumlah anakan, 2) penerapan macam dan dosis pupuk organik tidak berpengaruh terhadaap berat segar brangkasan, berat kering brangkasan, jumlah biji, berat biji, dan berat 100 biji, 3) macam dan dosis pupuk terbaik adalah MD2 (pupuk kandang dosis 400 g/polybag) karena dapat meningkatkan jumlah daun dan jumlah anakan.
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8

Huang, Ping, Georgiana Cheng, Haiyan Lu, Mark Aronica, Richard M. Ransohoff, and Lan Zhou. "Impaired respiratory function in mdx and mdx/utrn +/− mice." Muscle & Nerve 43, no. 2 (January 19, 2011): 263–67. http://dx.doi.org/10.1002/mus.21848.

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9

Wehmeyer, Jeffrey M. "MDX Health Digest." Medical Reference Services Quarterly 14, no. 2 (June 7, 1995): 53–60. http://dx.doi.org/10.1300/j115v14n02_05.

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10

Kempa, Martin. "Multidimensional Expressions (MDX)." Datenbank-Spektrum 11, no. 2 (June 23, 2011): 123–26. http://dx.doi.org/10.1007/s13222-011-0058-2.

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11

Sticklen, Jon, B. Chadrasekaran, J. W. Smith, and John Svirbely. "MDX-MYCIN: The MDX paradigm applied to the mycin domain." Computers & Mathematics with Applications 11, no. 5 (May 1985): 527–39. http://dx.doi.org/10.1016/0898-1221(85)90055-0.

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12

Morgan, Jennifer E., Gary R. Coulton, and Terence A. Partridge. "Mdx muscle grafts retain the mdx phenotype in normal hosts." Muscle & Nerve 12, no. 5 (May 1989): 401–9. http://dx.doi.org/10.1002/mus.880120511.

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13

Burns, David P., Sarah E. Drummond, Dearbhla Bolger, Amélie Coiscaud, Kevin H. Murphy, Deirdre Edge, and Ken D. O’Halloran. "N-acetylcysteine Decreases Fibrosis and Increases Force-Generating Capacity of mdx Diaphragm." Antioxidants 8, no. 12 (November 24, 2019): 581. http://dx.doi.org/10.3390/antiox8120581.

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Respiratory muscle weakness occurs due to dystrophin deficiency in Duchenne muscular dystrophy (DMD). The mdx mouse model of DMD shows evidence of impaired respiratory muscle performance with attendant inflammation and oxidative stress. We examined the effects of N-acetylcysteine (NAC) supplementation on respiratory system performance in mdx mice. Eight-week-old male wild type (n = 10) and mdx (n = 20) mice were studied; a subset of mdx (n = 10) received 1% NAC in the drinking water for 14 days. We assessed breathing, diaphragm, and external intercostal electromyogram (EMG) activities and inspiratory pressure during ventilatory and non-ventilatory behaviours. Diaphragm muscle structure and function, cytokine concentrations, glutathione status, and mRNA expression were determined. Diaphragm force-generating capacity was impaired in mdx compared with wild type. Diaphragm muscle remodelling was observed in mdx, characterized by increased muscle fibrosis, immune cell infiltration, and central myonucleation. NAC supplementation rescued mdx diaphragm function. Collagen content and immune cell infiltration were decreased in mdx + NAC compared with mdx diaphragms. The cytokines IL-1β, IL-6 and KC/GRO were increased in mdx plasma and diaphragm compared with wild type; NAC decreased systemic IL-1β and KC/GRO concentrations in mdx mice. We reveal that NAC treatment improved mdx diaphragm force-generating capacity associated with beneficial anti-inflammatory and anti-fibrotic effects. These data support the potential use of NAC as an adjunctive therapy in human dystrophinopathies.
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14

Stupka, Nicole, Jonathan D. Schertzer, Rhonda Bassel-Duby, Eric N. Olson, and Gordon S. Lynch. "Stimulation of calcineurin Aα activity attenuates muscle pathophysiology in mdx dystrophic mice." American Journal of Physiology-Regulatory, Integrative and Comparative Physiology 294, no. 3 (March 2008): R983—R992. http://dx.doi.org/10.1152/ajpregu.00375.2007.

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Calcineurin activation ameliorates the dystrophic pathology of hindlimb muscles in mdx mice and decreases their susceptibility to contraction damage. In mdx mice, the diaphragm is more severely affected than hindlimb muscles and more representative of Duchenne muscular dystrophy. The constitutively active calcineurin Aα transgene (CnAα) was overexpressed in skeletal muscles of mdx ( mdx CnAα*) mice to test whether muscle morphology and function would be improved. Contractile function of diaphragm strips and extensor digitorum longus and soleus muscles from adult mdx CnAα* and mdx mice was examined in vitro. Hindlimb muscles from mdx CnAα* mice had a prolonged twitch time course and were more resistant to fatigue. Because of a slower phenotype and a decrease in fiber cross-sectional area, normalized force was lower in fast- and slow-twitch muscles of mdx CnAα* than mdx mice. In the diaphragm, despite a slower phenotype and a ∼35% reduction in fiber size, normalized force was preserved. This was likely mediated by the reduction in the area of the diaphragm undergoing degeneration (i.e., mononuclear cell and connective and adipose tissue infiltration). The proportion of centrally nucleated fibers was reduced in mdx CnAα* compared with mdx mice, indicative of improved myofiber viability. In hindlimb muscles of mdx mice, calcineurin activation increased expression of markers of regeneration, particularly developmental myosin heavy chain isoform and myocyte enhancer factor 2A. Thus activation of the calcineurin signal transduction pathway has potential to ameliorate the mdx pathophysiology, especially in the diaphragm, through its effects on muscle degeneration and regeneration and endurance capacity.
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15

Capote, Joana, Marino DiFranco, and Julio L. Vergara. "Excitation-contraction coupling alterations in mdx and utrophin/dystrophin double knockout mice: a comparative study." American Journal of Physiology-Cell Physiology 298, no. 5 (May 2010): C1077—C1086. http://dx.doi.org/10.1152/ajpcell.00428.2009.

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The double knockout mouse for utrophin and dystrophin ( utr−/− /mdx) has been proposed to be a better model of Duchenne Muscular Dystrophy (DMD) than the mdx mouse because the former displays more similar muscle pathology to that of the DMD patients. In this paper the properties of action potentials (APs) and Ca2+ transients elicited by single and repetitive stimulation were studied to understand the excitation-contraction (EC) coupling alterations observed in muscle fibers from mdx and utr−/− /mdx mice. Based on the comparison of the AP durations with those of fibers from wild-type (WT) mice, fibers from both mdx and utr−/− /mdx mice could be divided in two groups: fibers with WT-like APs ( group 1) and fibers with significantly longer APs ( group 2). Although the proportion of fibers in group 2 was larger in utr−/− /mdx (36%) than in mdx mice (27%), the Ca2+ release elicited by single stimulation was found to be similarly depressed (32–38%) in utr−/− /mdx and mdx fibers compared with WT counterparts regardless of the fiber's group. Stimulation at 100 Hz revealed that, with the exception of those from utr−/− /mdx mice, group 1 fibers were able to sustain Ca2+ release for longer than group 2 fibers, which displayed an abrupt limitation even at the onset of the train. The differences in behavior between fibers in groups 1 and 2 became almost unnoticeable at 50 Hz stimulation. In general, fibers from utr−/− /mdx mice seem to display more persistent alterations in the EC coupling than those observed in the mdx model.
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16

Rouger, Karl, Martine Le Cunff, Marja Steenman, Marie-Claude Potier, Nathalie Gibelin, Claude A. Dechesne, and Jean J. Leger. "Global/temporal gene expression in diaphragm and hindlimb muscles of dystrophin-deficient (mdx) mice." American Journal of Physiology-Cell Physiology 283, no. 3 (September 1, 2002): C773—C784. http://dx.doi.org/10.1152/ajpcell.00112.2002.

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The mdx mouse is a model for human Duchenne muscular dystrophy (DMD), an X-linked degenerative disease of skeletal muscle tissue characterized by the absence of the dystrophin protein. The mdx mice display a much milder phenotype than DMD patients. After the first week of life when all mdx muscles evolve like muscles of young DMD patients, mdx hindlimb muscles substantially compensate for the lack of dystrophin, whereas mdx diaphragm muscle becomes progressively affected by the disease. We used cDNA microarrays to compare the expression profile of 1,082 genes, previously selected by a subtractive method, in control and mdx hindlimb and diaphragm muscles at 12 time points over the first year of the mouse life. We determined that 1) the dystrophin gene defect induced marked expression remodeling of 112 genes encoding proteins implicated in diverse muscle cell functions and 2) two-thirds of the observed transcriptomal anomalies differed between adult mdx hindlimb and diaphragm muscles. Our results showed that neither mdx diaphram muscle nor mdx hindlimb muscles evolve entirely like the human DMD muscles. This finding should be taken under consideration for the interpretation of future experiments using mdx mice as a model for therapeutic assays.
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17

Lynch, Gordon S., Jill A. Rafael, Jeffrey S. Chamberlain, and John A. Faulkner. "Contraction-induced injury to single permeabilized muscle fibers from mdx, transgenic mdx, and control mice." American Journal of Physiology-Cell Physiology 279, no. 4 (October 1, 2000): C1290—C1294. http://dx.doi.org/10.1152/ajpcell.2000.279.4.c1290.

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Muscle fibers of mdx mice that lack dystrophin are more susceptible to contraction-induced injury, particularly when stretched. In contrast, transgenic mdx (tg -mdx) mice, which overexpress dystrophin, show no morphological or functional signs of dystrophy. Permeabilization disrupts the sarcolemma of fibers from muscles of mdx, tg- mdx, and control mice. We tested the null hypothesis stating that, after single stretches of maximally activated single permeabilized fibers, force deficits do not differ among fibers from extensor digitorum longus muscles of mdx, tg -mdx, or control mice. Fibers were maximally activated by Ca2+ (pCa 4.5) and then stretched through strains of 10%, 20%, or 30% of fiber length ( L f) at a velocity of 0.5 L f/s. Immediately after each strain, the force deficits were not different for fibers from each of the three groups of mice. When collated with studies of membrane-intact fibers in whole muscles of mdx, tg -mdx, and control mice, these results indicate that dystrophic symptoms do not arise from factors within myofibrils but, rather, from disruption of the sarcolemmal integrity that normally provides protection from contraction-induced injury.
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18

Williams, Iwan A., and David G. Allen. "The role of reactive oxygen species in the hearts of dystrophin-deficient mdx mice." American Journal of Physiology-Heart and Circulatory Physiology 293, no. 3 (September 2007): H1969—H1977. http://dx.doi.org/10.1152/ajpheart.00489.2007.

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Duchenne muscular dystrophy (DMD) is caused by deficiency of the cytoskeletal protein dystrophin. Oxidative stress is thought to contribute to the skeletal muscle damage in DMD; however, little is known about the role of oxidative damage in the pathogenesis of the heart failure that occurs in DMD patients. The dystrophin-deficient ( mdx) mouse is an animal model of DMD that also lacks dystrophin. The current study investigates the role of the antioxidant N-acetylcysteine (NAC) on mdx cardiomyocyte function, Ca2+ handling, and the cardiac inflammatory response. Treated mice received 1% NAC in their drinking water for 6 wk. NAC had no effect on wild-type (WT) mice. Immunohistochemistry experiments revealed that mdx mice had increased dihydroethidine (DHE) staining, an indicator of superoxide production; NAC-treatment reduced DHE staining in mdx hearts. NAC treatment attenuated abnormalities in mdx cardiomyocyte Ca2+ handling. Mdx cardiomyocytes had decreased fractional shortening and decreased Ca2+ sensitivity; NAC treatment returned mdx fractional shortening to WT values but did not affect the Ca2+ sensitivity. Immunohistochemistry experiments revealed that mdx hearts had increased levels of collagen type III and the macrophage-specific protein, CD68; NAC-treatment returned collagen type III and CD68 expression close to WT values. Finally, mdx hearts had increased NADPH oxidase activity, suggesting it could be a possible source of increased reactive oxygen species in mdx mice. This study is the first to demonstrate that oxidative damage may be involved in the pathogenesis of the heart failure that occurs in mdx mice. Therapies designed to reduce oxidative damage might be beneficial to DMD patients with heart failure.
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19

Mázala, Davi A. G., Stephen J. P. Pratt, Dapeng Chen, Jeffery D. Molkentin, Richard M. Lovering, and Eva R. Chin. "SERCA1 overexpression minimizes skeletal muscle damage in dystrophic mouse models." American Journal of Physiology-Cell Physiology 308, no. 9 (May 1, 2015): C699—C709. http://dx.doi.org/10.1152/ajpcell.00341.2014.

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Duchenne muscular dystrophy (DMD) is characterized by progressive muscle wasting secondary to repeated muscle damage and inadequate repair. Elevations in intracellular free Ca2+ have been implicated in disease progression, and sarcoplasmic/endoplasmic reticulum Ca2+-ATPase 1 (SERCA1) overexpression has been shown to ameliorate the dystrophic phenotype in mdx mice. The purpose of this study was to assess the effects of SERCA1 overexpression in the more severe mdx/Utr−/− mouse model of DMD. Mice overexpressing SERCA1 were crossed with mdx/Utr+/− mice to generate mdx/Utr−/−/+SERCA1 mice and compared with wild-type (WT), WT/+SERCA1, mdx/+SERCA1, and genotype controls. Mice were assessed at ∼12 wk of age for changes in Ca2+ handling, muscle mass, quadriceps torque, markers of muscle damage, and response to repeated eccentric contractions. SERCA1-overexpressing mice had a two- to threefold increase in maximal sarcoplasmic reticulum Ca2+-ATPase activity compared with WT which was associated with normalization in body mass for both mdx/+SERCA1 and mdx/Utr−/−/+SERCA1. Torque deficit in the quadriceps after eccentric injury was 2.7-fold greater in mdx/Utr−/− vs. WT mice, but only 1.5-fold greater in mdx/Utr−/−/+SERCA1 vs. WT mice, an attenuation of 44%. Markers of muscle damage (% centrally nucleated fibers, necrotic area, and serum creatine kinase levels) were higher in both mdx and mdx/Utr−/− vs. WT, and all were attenuated by overexpression of SERCA1. These data indicate that SERCA1 overexpression ameliorates functional impairments and cellular markers of damage in a more severe mouse model of DMD. These findings support targeting intracellular Ca2+ control as a therapeutic approach for DMD.
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20

Vannucchi, M. G., R. Garella, G. Cipriani, and M. C. Baccari. "Relaxin counteracts the altered gastric motility of dystrophic (mdx) mice: functional and immunohistochemical evidence for the involvement of nitric oxide." American Journal of Physiology-Endocrinology and Metabolism 300, no. 2 (February 2011): E380—E391. http://dx.doi.org/10.1152/ajpendo.00375.2010.

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Impaired gastric motility ascribable to a defective nitric oxide (NO) production has been reported in dystrophic (mdx) mice. Since relaxin upregulates NO biosynthesis, its effects on the motor responses and NO synthase (NOS) expression in the gastric fundus of mdx mice were investigated. Mechanical responses of gastric strips were recorded via force displacement transducers. Evaluation of the three NOS isoforms was performed by immunohistochemistry and Western blot. Wild-type (WT) and mdx mice were distributed into three groups: untreated, relaxin pretreated, and vehicle pretreated. In strips from both untreated and vehicle-pretreated animals, electrical field stimulation (EFS) elicited contractile responses that were greater in mdx than in WT mice. In carbachol-precontracted strips, EFS induced fast relaxant responses that had a lower amplitude in mdx than in WT mice. Only in the mdx mice did relaxin depress the amplitude of the neurally induced excitatory responses and increase that of the inhibitory ones. In the presence of l-NNA, relaxin was ineffective. In relaxin-pretreated mdx mice, the amplitude of the EFS-induced contractile responses was decreased and that of the fast relaxant ones was increased compared with untreated mdx animals. Responses to methacholine or papaverine did not differ among preparations and were not influenced by relaxin. Immunohistochemistry and Western blotting showed a significant decrease in neuronal NOS expression and content in mdx compared with WT mice, which was recovered in the relaxin-pretreated mdx mice. The results suggest that relaxin is able to counteract the altered contractile and relaxant responses in the gastric fundus of mdx mice by upregulating nNOS expression.
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Ueda, Junpei, and Shigeyoshi Saito. "Evaluation of Cardiac Function in Young Mdx Mice Using MRI with Feature Tracking and Self-Gated Magnetic Resonance Cine Imaging." Diagnostics 13, no. 8 (April 19, 2023): 1472. http://dx.doi.org/10.3390/diagnostics13081472.

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This study aimed to evaluate cardiac function in a young mouse model of Duchenne muscular dystrophy (mdx) using cardiac magnetic resonance imaging (MRI) with feature tracking and self-gated magnetic resonance cine imaging. Cardiac function was evaluated in mdx and control mice (C57BL/6JJmsSlc mice) at 8 and 12 weeks of age. Preclinical 7-T MRI was used to capture short-axis, longitudinal two-chamber view and longitudinal four-chamber view cine images of mdx and control mice. Strain values were measured and evaluated from cine images acquired using the feature tracking method. The left ventricular ejection fraction was significantly less (p < 0.01 each) in the mdx group at both 8 (control, 56.6 ± 2.3% mdx, 47.2 ± 7.4%) and 12 weeks (control, 53.9 ± 3.3% mdx, 44.1 ± 2.7%). In the strain analysis, all strain value peaks were significantly less in mdx mice, except for the longitudinal strain of the four-chamber view at both 8 and 12 weeks of age. Strain analysis with feature tracking and self-gated magnetic resonance cine imaging is useful for assessing cardiac function in young mdx mice.
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22

Dupont-Versteegden, E. E., R. J. McCarter, and M. S. Katz. "Voluntary exercise decreases progression of muscular dystrophy in diaphragm of mdx mice." Journal of Applied Physiology 77, no. 4 (October 1, 1994): 1736–41. http://dx.doi.org/10.1152/jappl.1994.77.4.1736.

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Effects of voluntary wheel running on contractile properties of diaphragm (DIA) and soleus (SOL) of dystrophic (mdx) and control (C57BL/10SNJ) mice were evaluated. In particular, we tested the hypothesis that daily voluntary running is not deleterious to muscle function in mdx mice. Both groups of mice ran extensively (control mice approximately 7 km/day, mdx mice approximately 5 km/day). Exercise increased maximal specific tetanus tension of mdx DIA from 1.02 +/- 0.04 to 1.33 +/- 0.06 kg/cm2 but did not restore it to the control level (2.55 +/- 0.17 kg/cm2). Maximal tetanus tension of sedentary mdx SOL (2.41 +/- 0.17 kg/cm2) was reduced compared with control (3.10 +/- 0.15 kg/cm2) and was not altered by running activity. Optimal length was significantly lower in DIA of mdx mice, and exercise did not change this. Fatigability and contractile properties of muscles measured in vitro were not altered by running activity with the exception of increased contraction time in mdx DIA. In conclusion, extensive wheel running is not deleterious to muscle function in mdx mice contrary to predictions of the “work overload” theory of muscular dystrophy. Rather, this exercise is beneficial for active tension generation of mdx DIA, the muscle most closely resembling muscles of patients with Duchenne muscular dystrophy.
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Lynch, G. S., J. A. Rafael, R. T. Hinkle, N. M. Cole, J. S. Chamberlain, and J. A. Faulkner. "Contractile properties of diaphragm muscle segments from old mdx and old transgenic mdx mice." American Journal of Physiology-Cell Physiology 272, no. 6 (June 1, 1997): C2063—C2068. http://dx.doi.org/10.1152/ajpcell.1997.272.6.c2063.

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Diaphragm muscles of young (4- to 6-mo-old) mdx mice show severe fiber necrosis and have normalized forces and powers 60 and 46% of the values for control C57BL/10 mice. In contrast, microinjection of mdx mouse embryos with a truncated dystrophin minigene has produced young transgenic mdx (tg-mdx) mice with a level of dystrophin expression and structural and functional properties of diaphragm muscle strips measured in vitro not different from those of control mice. Whether dystrophin expression and functional corrections persist for the life span of these animals is not know. We tested the null hypothesis that, in old (24 mo) tg-mdx mice, dystrophin expression is adequate and diaphragm muscle strips have forces and powers not different from values for diaphragm muscle strips from young tg-mdx mice or control mice. Compared with control values, diaphragm muscle strips from old mdx mice had normalized forces and powers of 48 and 31%, respectively. Expression of dystrophin persisted in diaphragm muscles of old tg-mdx mice, and functional properties were not different from diaphragm muscles of young tg-mdx or young or old control mice. These results suggest that, with a transgenic animal approach, dystrophin expression and functional corrections persist for the life span of the animals.
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24

Gartz, Melanie, Margaret Haberman, Mariah J. Prom, Margaret J. Beatka, Jennifer L. Strande, and Michael W. Lawlor. "A Long-Term Study Evaluating the Effects of Nicorandil Treatment on Duchenne Muscular Dystrophy-Associated Cardiomyopathy in mdx Mice." Journal of Cardiovascular Pharmacology and Therapeutics 27 (January 1, 2022): 107424842210886. http://dx.doi.org/10.1177/10742484221088655.

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Background: Duchenne muscular dystrophy (DMD) is a neuromuscular disease caused by dystrophin gene mutations affecting striated muscle. Due to advances in skeletal muscle treatment, cardiomyopathy has emerged as a leading cause of death. Previously, nicorandil, a drug with antioxidant and nitrate-like properties, ameliorated cardiac damage and improved cardiac function in young, injured mdx mice. Nicorandil mitigated damage by stimulating antioxidant activity and limiting pro-oxidant expression. Here, we examined whether nicorandil was similarly cardioprotective in aged mdx mice. Methods and Results: Nicorandil (6 mg/kg) was given over 15 months. Echocardiography of mdx mice showed some functional defects at 12 months compared to wild-type (WT) mice, but not at 15 months. Disease manifestation was evident in mdx mice via treadmill assays and survival, but not open field and grip strength assays. Cardiac levels of SOD2 and NOX4 were decreased in mdx vs. WT. Nicorandil increased survival in mdx but did not alter cardiac function, fibrosis, diaphragm function or muscle fatigue. Conclusions: In contrast to our prior work in young, injured mdx mice, nicorandil did not exert cardioprotective effects in 15 month aged mdx mice. Discordant findings may be explained by the lack of cardiac disease manifestation in aged mdx mice compared to WT, whereas significant cardiac dysfunction was previously seen with the sub-acute injury in young mice. Therefore, we are not able to conclude any cardioprotective effects with long-term nicorandil treatment in aging mdx mice.
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25

Barker, Robert, Chris van der Poel, Deanna Horvath, and Robyn Murphy. "Taurine and Methylprednisolone Administration at Close Proximity to the Onset of Muscle Degeneration Is Ineffective at Attenuating Force Loss in the Hind-Limb of 28 Days Mdx Mice." Sports 6, no. 4 (September 30, 2018): 109. http://dx.doi.org/10.3390/sports6040109.

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An increasing number of studies have shown supplementation with the amino acid taurine to have promise in ameliorating dystrophic symptoms in the mdx mouse model of Duchenne Muscular Dystrophy (DMD). Here we build on this limited body of work by investigating the efficacy of supplementing mdx mice with taurine postnatally at a time suggestive of when dystrophic symptoms would begin to manifest in humans, and when treatments would likely begin. Mdx mice were given either taurine (mdx tau), the steroid alpha methylprednisolone (PDN), or tau + PDN (mdx tau + PDN). Taurine (2.5% wt/vol) enriched drinking water was given from 14 days and PDN (1 mg/kg daily) from 18 days. Wild-type (WT, C57BL10/ScSn) mice were used as a control to mdx mice to represent healthy tissue. In the mdx mouse, peak damage occurs at 28 days, and in situ assessment of contractile characteristics showed that taurine, PDN, and the combined taurine + PDN treatment was ineffective at attenuating the force loss experienced by mdx mice. Given the benefits of taurine as well as methylprednisolone reported previously, when supplemented at close proximity to the onset of severity muscle degeneration these benefits are no longer apparent.
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26

Kravtsova, Violetta V., Elena V. Bouzinova, Alexander V. Chibalin, Vladimir V. Matchkov, and Igor I. Krivoi. "Isoform-specific Na,K-ATPase and membrane cholesterol remodeling in motor endplates in distinct mouse models of myodystrophy." American Journal of Physiology-Cell Physiology 318, no. 5 (May 1, 2020): C1030—C1041. http://dx.doi.org/10.1152/ajpcell.00453.2019.

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Na,K-ATPase is a membrane transporter that is critically important for skeletal muscle function. Mdx and Bla/J mice are the experimental models of Duchenne muscular dystrophy and dysferlinopathy that are known to differ in the molecular mechanism of the pathology. This study examines the function of α1- and α2-Na,K-ATPase isozymes in respiratory diaphragm and postural soleus muscles from mdx and Bla/J mice compared with control С57Bl/6 mice. In diaphragm muscles, the motor endplate structure was severely disturbed (manifested by defragmentation) in mdx mice only. The endplate membrane of both Bla/J and mdx mice was depolarized due to specific loss of the α2-Na,K-ATPase electrogenic activity and its decreased membrane abundance. Total FXYD1 subunit (modulates Na,K-ATPase activity) abundance was decreased in both mouse models. However, the α2-Na,K-ATPase protein content as well as mRNA expression were specifically and significantly reduced only in mdx mice. The endplate membrane cholesterol redistribution was most pronounced in mdx mice. Soleus muscles from Bla/J and mdx mice demonstrated reduction of the α2-Na,K-ATPase membrane abundance and mRNA expression similar to the diaphragm muscles. In contrast to diaphragm, the α2-Na,K-ATPase protein content was altered in both Bla/J and mdx mice; membrane cholesterol re-distribution was not observed. Thus, the α2-Na,K-ATPase is altered in both Bla/J and mdx mouse models of chronic muscle pathology. However, despite some similarities, the α2-Na,K-ATPase and cholesterol abnormalities are more pronounced in mdx mice.
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27

Gonzalez, Daniel R., Adriana V. Treuer, Guillaume Lamirault, Vera Mayo, Yenong Cao, Raul A. Dulce, and Joshua M. Hare. "NADPH oxidase-2 inhibition restores contractility and intracellular calcium handling and reduces arrhythmogenicity in dystrophic cardiomyopathy." American Journal of Physiology-Heart and Circulatory Physiology 307, no. 5 (September 1, 2014): H710—H721. http://dx.doi.org/10.1152/ajpheart.00890.2013.

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Duchenne muscular dystrophy may affect cardiac muscle, producing a dystrophic cardiomyopathy in humans and the mdx mouse. We tested the hypothesis that oxidative stress participates in disrupting calcium handling and contractility in the mdx mouse with established cardiomyopathy. We found increased expression (fivefold) of the NADPH oxidase (NOX) 2 in the mdx hearts compared with wild type, along with increased superoxide production. Next, we tested the impact of NOX2 inhibition on contractility and calcium handling in isolated cardiomyocytes. Contractility was decreased in mdx myocytes compared with wild type, and this was restored toward normal by pretreating with apocynin. In addition, the amplitude of evoked intracellular Ca2+ concentration transients that was diminished in mdx myocytes was also restored with NOX2 inhibition. Total sarcoplasmic reticulum (SR) Ca2+ content was reduced in mdx hearts and normalized by apocynin treatment. Additionally, NOX2 inhibition decreased the production of spontaneous diastolic calcium release events and decreased the SR calcium leak in mdx myocytes. In addition, nitric oxide (NO) synthase 1 (NOS-1) expression was increased eightfold in mdx hearts compared with wild type. Nevertheless, cardiac NO production was reduced. To test whether this paradox implied NOS-1 uncoupling, we treated cardiac myocytes with exogenous tetrahydrobioterin, along with the NOX inhibitor VAS2870. These agents restored NO production and phospholamban phosphorylation in mdx toward normal. Together, these results demonstrate that, in mdx hearts, NOX2 inhibition improves the SR calcium handling and contractility, partially by recoupling NOS-1. These findings reveal a new layer of nitroso-redox imbalance in dystrophic cardiomyopathy.
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28

MacLennan, P. A., and R. H. T. Edwards. "Protein turnover is elevated in muscle of mdx mice in vivo." Biochemical Journal 268, no. 3 (June 15, 1990): 795–97. http://dx.doi.org/10.1042/bj2680795.

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mdx mice lack the protein dystrophin, the absence of which causes Duchenne muscular dystrophy in humans. To examine how mdx mice maintain muscle mass despite dystrophin deficiency, we measured protein turnover rates in muscles of mdx and wild-type (C57BL/10) mice in vivo. At all ages studied, rates of muscle protein synthesis and degradation were higher in mdx than in C57BL/10 mice.
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29

Hakim, Chady H., Robert W. Grange, and Dongsheng Duan. "The passive mechanical properties of the extensor digitorum longus muscle are compromised in 2- to 20-mo-old mdx mice." Journal of Applied Physiology 110, no. 6 (June 2011): 1656–63. http://dx.doi.org/10.1152/japplphysiol.01425.2010.

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Muscle rigidity and myotendinous junction (MTJ) deficiency contribute to immobilization in Duchenne muscular dystrophy (DMD), a lethal disease caused by the absence of dystrophin. However, little is known about the muscle passive properties and MTJ strength in a diseased muscle. Here, we hypothesize that dystrophin-deficient muscle pathology renders skeletal muscle stiffer and MTJ weaker. To test our hypothesis, we examined the passive properties of an intact noncontracting muscle-tendon unit in mdx mice, a mouse model for DMD. The extensor digitorum longus (EDL) muscle-tendon preparations of 2-, 6-, 14-, and 20-mo-old mdx and normal control mice were strained stepwisely from 110% to 160% of the muscle optimal length. The stress-strain response and failure position were analyzed. In support of our hypothesis, the mdx EDL preparation consistently developed higher stress before muscle failure. Postfailure stresses decreased dramatically in mdx but not normal preparations. Further, mdx showed a significantly faster stress relaxation rate. Consistent with stress-strain assay results, we observed significantly higher fibrosis in mdx muscle. In 2- and 6-mo-old mdx and 20-mo-old BL10 mice failure occurred within the muscle (2- to 14-mo-old BL10 preparations did not fail). Interestingly, in ≥14-mo-old mdx mice the failure site shifted toward the MTJ. Electron microscopy revealed substantial MTJ degeneration in aged but not young mdx mice. In summary, our results suggest that the passive properties of the EDL muscle and the strength of MTJ are compromised in mdx in an age-dependent manner. These findings offer new insights in studying DMD pathogenesis and developing novel therapies.
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Coirault, Catherine, Bernadette Pignol, Racquel N. Cooper, Gillian Butler-Browne, Pierre-Etienne Chabrier, and Yves Lecarpentier. "Severe muscle dysfunction precedes collagen tissue proliferation in mdx mouse diaphragm." Journal of Applied Physiology 94, no. 5 (May 1, 2003): 1744–50. http://dx.doi.org/10.1152/japplphysiol.00989.2002.

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After extensive necrosis, progressive diaphragm muscle weakness in the mdx mouse is thought to reflect progressive replacement of contractile tissue by fibrosis. However, little has been documented on diaphragm muscle performance at the stage at which necrosis and fibrosis are limited. Diaphragm morphometric characteristics, muscle performance, and cross-bridge (CB) properties were investigated in 6-wk-old control (C) and mdx mice. Compared with C, maximum tetanic tension and shortening velocity were 37 and 32% lower, respectively, in mdx mice (each P < 0.05). The total number of active CB per millimeter squared (13.0 ± 1.2 vs. 18.4 ± 1.7 × 109/mm2, P < 0.05) and the CB elementary force (8.0 ± 0.2 vs. 9.0 ± 0.1 pN, P < 0.01) were lower in mdx than in C. The time cycle duration was lower in mdx than in C (127 ± 18 vs. 267 ± 61 ms, P < 0.05). Percentages of fiber necrosis represented 2.8 ± 0.6% of the total muscle fibers, and collagen surface area occupied 3.6 ± 0.7% in mdx diaphragm. Our results pointed to severe muscular dysfunction in mdx mouse diaphragm, despite limited necrotic and fibrotic lesions.
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31

Yoshida, Mizuko, Akira Yonetani, Toshihiro Shirasaki, and Keiji Wada. "Dietary NaCl supplementation prevents muscle necrosis in a mouse model of Duchenne muscular dystrophy." American Journal of Physiology-Regulatory, Integrative and Comparative Physiology 290, no. 2 (February 2006): R449—R455. http://dx.doi.org/10.1152/ajpregu.00684.2004.

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The mdx mouse is an animal model for Duchenne muscular dystrophy. Mdx mice fed a 12% NaCl diet from birth up to 20 days of age (mdx-Na mice) had an ∼50% reduction in serum creatine kinase (CK) activity compared with mdx mice fed a standard diet. Most notably, necrotic fibers in tibialis anterior (TA) muscle of mdx-Na mice were reduced by 99% and were similar in control mice. These mdx mice displayed significantly elevated blood Ca2+ and Na+ levels, while the total calcium content of their TA muscle was reduced to the level of control mice. In addition, mdx-Na mice had elevated zinc and magnesium contents in their TA muscle. These results suggest that elevated serum Na+ leads to Ca2+ extrusion from muscle via the Na+/Ca2+ exchanger causing a decrease in intracellular Ca2+ levels and an increase in blood Ca2+ levels. Extracellular Ca2+ and, in addition, Zn2+ and Mg2+ might also contribute to the stabilization of the cell membrane. Other possibilities explaining the surprisingly efficacious beneficial effect of dietary sodium exist and are discussed.
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32

Suelves, Mònica, Berta Vidal, Antonio L. Serrano, Marc Tjwa, Josep Roma, Roser López-Alemany, Aernout Luttun, et al. "uPA deficiency exacerbates muscular dystrophy in MDX mice." Journal of Cell Biology 178, no. 6 (September 4, 2007): 1039–51. http://dx.doi.org/10.1083/jcb.200705127.

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Duchenne muscular dystrophy (DMD) is a fatal and incurable muscle degenerative disorder. We identify a function of the protease urokinase plasminogen activator (uPA) in mdx mice, a mouse model of DMD. The expression of uPA is induced in mdx dystrophic muscle, and the genetic loss of uPA in mdx mice exacerbated muscle dystrophy and reduced muscular function. Bone marrow (BM) transplantation experiments revealed a critical function for BM-derived uPA in mdx muscle repair via three mechanisms: (1) by promoting the infiltration of BM-derived inflammatory cells; (2) by preventing the excessive deposition of fibrin; and (3) by promoting myoblast migration. Interestingly, genetic loss of the uPA receptor in mdx mice did not exacerbate muscular dystrophy in mdx mice, suggesting that uPA exerts its effects independently of its receptor. These findings underscore the importance of uPA in muscular dystrophy.
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33

Lovering, Richard M., Luke Michaelson, and Christopher W. Ward. "Malformedmdxmyofibers have normal cytoskeletal architecture yet altered EC coupling and stress-induced Ca2+signaling." American Journal of Physiology-Cell Physiology 297, no. 3 (September 2009): C571—C580. http://dx.doi.org/10.1152/ajpcell.00087.2009.

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Skeletal muscle function is dependent on its highly regular structure. In studies of dystrophic ( dy/dy) mice, the proportion of malformed myofibers decreases after prolonged whole muscle stimulation, suggesting that the malformed myofibers are more prone to injury. The aim of this study was to assess morphology and to measure excitation-contraction (EC) coupling (Ca2+transients) and susceptibility to osmotic stress (Ca2+sparks) of enzymatically isolated muscle fibers of the extensor digitorum longus (EDL) and flexor digitorum brevis (FDB) muscles from young (2–3 mo) and old (8–9 mo) mdx and age-matched control mice (C57BL10). In young mdx EDL, 6% of the myofibers had visible malformations (i.e., interfiber splitting, branched ends, midfiber appendages). In contrast, 65% of myofibers in old mdx EDL contained visible malformations. In the mdx FDB, malformation occurred in only 5% of young myofibers and 11% of old myofibers. Age-matched control mice did not display the altered morphology of mdx muscles. The membrane-associated and cytoplasmic cytoskeletal structures appeared normal in the malformed mdx myofibers. In mdx FDBs with significantly branched ends, an assessment of global, electrically evoked Ca2+signals (indo-1PE-AM) revealed an EC coupling deficit in myofibers with significant branching. Interestingly, peak amplitude of electrically evoked Ca2+release in the branch of the bifurcated mdx myofiber was significantly decreased compared with the trunk of the same myofiber. No alteration in the basal myoplasmic Ca2+concentration (i.e., indo ratio) was seen in malformed vs. normal mdx myofibers. Finally, osmotic stress induced the occurrence of Ca2+sparks to a greater extent in the malformed portions of myofibers, which is consistent with deficits in EC coupling control. In summary, our data show that aging mdx myofibers develop morphological malformations. These malformations are not associated with gross disruptions in cytoskeletal or t-tubule structure; however, alterations in myofiber Ca2+signaling are evident.
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34

Vajda, Z., M. Pedersen, T. Doczi, E. Sulyok, and S. Nielsen. "Studies of mdx mice." Neuroscience 129, no. 4 (January 2004): 991–96. http://dx.doi.org/10.1016/j.neuroscience.2004.08.055.

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35

Desjardins, Philippe. "Microanalyte MDx Goes Mainstream." Genetic Engineering & Biotechnology News 37, no. 6 (March 15, 2017): 20–22. http://dx.doi.org/10.1089/gen.37.06.13.

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36

MacLennan, P. A., A. McArdle, and R. H. Edwards. "Effects of calcium on protein turnover of incubated muscles from mdx mice." American Journal of Physiology-Endocrinology and Metabolism 260, no. 4 (April 1, 1991): E594—E598. http://dx.doi.org/10.1152/ajpendo.1991.260.4.e594.

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Mdx mice have a genetic defect similar to that which causes Duchenne muscular dystrophy in humans. The influence of calcium on muscle protein metabolism of mdx and wild type (C57BL/10) mice was examined in vitro. Incubation of mdx muscles in a medium containing calcium at a concentration of 2.0 mM (but not 0.2 mM) resulted in proteolytic rates that were greater than those of C57BL/10 muscles. At 2.0 mM extracellular calcium, mdx muscle proteolysis was attenuated by thiol protease inhibitors but not by the weak base methylamine. Protein synthetic rates were higher in incubated mdx muscles than in incubated C57BL/10 muscles, but no effect of extracellular calcium concentration was observed in either strain. These data suggest that mdx mice have an abnormality of muscle calcium handling, which results in activation of nonlysosomal proteolytic processes but does not exert acute effects on protein synthetic rate.
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37

Rezvani, M., E. Cafarelli, and D. A. Hood. "Performance and excitability of mdx mouse muscle at 2, 5, and 13 wk of age." Journal of Applied Physiology 78, no. 3 (March 1, 1995): 961–67. http://dx.doi.org/10.1152/jappl.1995.78.3.961.

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Dystrophin is a 427-kDa protein localized adjacent to the sarcolemma in skeletal muscle. Its physiological role remains uncertain, although its absence is known to cause muscular dystrophy. In this study, the function of dystrophin was investigated using the dystrophin-deficient mdx mouse. Control and mdx animals at 2, 5, and 13 wk of age (n = 8–11/age) were compared to evaluate in situ gastrocnemius-plantaris-soleus muscle contractile, endurance, and excitability properties at nondegenerated, degenerated, and regenerated stages, respectively. Twitch and tetanic tensions expressed per gram of muscle mass were lower in mdx muscle only at 5 wk. Fatigue produced during successive contractions at 2, 10, and 20 Hz did not differ between the two groups at 2 and 5 wk but was lower in mdx muscle at 13 wk. This was not attributed to differences in mitochondria, since cytochrome-c oxidase activity was similar in mdx and control muscle. Contractile properties of control and mdx muscle became faster with age, and at 13 wk the time to peak twitch tension was shorter in mdx muscle relative to control, whereas the half-relaxation times did not differ. Mass action potential area (M wave), an index of muscle excitability, was not significantly different between mdx and control muscle at 2 or 5 wk but was greater in mdx muscle at 13 wk. Thus, in this weight-bearing muscle group, the lack of dystrophin has only a moderate impact in modifying muscle function relative to contractile properties, fatigability, or excitability.(ABSTRACT TRUNCATED AT 250 WORDS)
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38

Plant, David R., and Gordon S. Lynch. "Depolarization-induced contraction and SR function in mechanically skinned muscle fibers from dystrophic mdx mice." American Journal of Physiology-Cell Physiology 285, no. 3 (September 2003): C522—C528. http://dx.doi.org/10.1152/ajpcell.00369.2002.

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Dystrophin is absent in muscle fibers of patients with Duchenne muscular dystrophy (DMD) and in muscle fibers from the mdx mouse, an animal model of DMD. Disrupted excitation-contraction (E-C) coupling has been postulated to be a functional consequence of the lack of dystrophin, although the evidence for this is not entirely clear. We used mechanically skinned fibers (with a sealed transverse tubular system) prepared from fast extensor digitorum longus muscles of wild-type control and dystrophic mdx mice to test the hypothesis that dystrophin deficiency would affect the depolarization-induced contractile response (DICR) and sarcoplasmic reticulum (SR) function. DICR was similar in muscle fibers from mdx and control mice, indicating normal voltage regulation of Ca2+ release. Nevertheless, rundown of DICR (<50% of initial) was reached more rapidly in fibers from mdx than control mice [control: 32 ± 5 depolarizations ( n = 14 fibers) vs. mdx: 18 ± 1 depolarizations ( n = 7) before rundown, P < 0.05]. The repriming rate for DICRs was decreased in fibers from mdx mice, with lower submaximal DICR observed after 5, 10, and 20 s of repriming compared with fibers from control mice ( P < 0.05). SR Ca2+ reloading was not different in fibers from control and mdx mice, and no difference was observed in SR Ca2+ leak. Caffeine (2–7 mM)-induced contraction was diminished in fibers from mdx mice compared with control ( P < 0.05), indicating depressed SR Ca2+ release channel activity. Our findings indicate that fast fibers from mdx mice exhibit some impairment in the events mediating E-C coupling and SR Ca2+ release channel activity.
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39

Ren, Xiaoyu, Hongyang Xu, Robert G. Barker, Graham D. Lamb, and Robyn M. Murphy. "Elevated MMP2 abundance and activity in mdx mice are alleviated by prenatal taurine supplementation." American Journal of Physiology-Cell Physiology 318, no. 6 (June 1, 2020): C1083—C1091. http://dx.doi.org/10.1152/ajpcell.00437.2019.

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Duchenne muscular dystrophy (DMD) is a severe, progressive muscle-wasting disorder that leads to early death. The mdx mouse is a naturally occurring mutant model for DMD. It lacks dystrophin and displays peak muscle cell necrosis at ~28 days (D28), but in contrast to DMD, mdx mice experience muscle regeneration by D70. We hypothesized that matrix metalloproteinase-2 (MMP2) and/or MMP9 play key roles in the degeneration/regeneration phases in mdx mice. MMP2 abundance in muscle homogenates, measured by calibrated Western blotting, and activity, measured by zymogram, were lower at D70 compared with D28 in both mdx and wild-type (WT) mice. Importantly, MMP2 abundance was higher in both D28 and D70 mdx mice than in age-matched WT mice. The higher MMP2 abundance was not due to infiltrating macrophages, because MMP2 content was still higher in isolated muscle fibers where most macrophages had been removed. Prenatal supplementation with the amino acid taurine, which improved muscle strength in D28 mdx mice, produced approximately twofold lower MMP2 activity, indicating that increased MMP2 abundance is not required when muscle damage is attenuated. There was no difference in MMP9 abundance between age-matched WT and mdx mice ( P > 0.05). WT mice displayed decreased MMP9 abundance as they aged. While MMP9 may have a role during age-related skeletal muscle growth, it does not appear essential for degeneration/regeneration cycles in the mdx mouse. Our findings indicate that MMP2 plays a more active role than MMP9 in the degenerative phases of muscle fibers in D28 mdx mice.
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40

Hayes, Alan, and David A. Williams. "Contractile function and low-intensity exercise effects of old dystrophic (mdx) mice." American Journal of Physiology-Cell Physiology 274, no. 4 (April 1, 1998): C1138—C1144. http://dx.doi.org/10.1152/ajpcell.1998.274.4.c1138.

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Old mdx mice display a severe myopathy almost identical to Duchenne’s muscular dystrophy. This study examined the contractile properties of old mdxmuscles and investigated any effects of low-intensity exercise. Isometric contractile properties of the extensor digitorum longus (EDL) and soleus muscles were tested in adult (8–10 mo) and old (24 mo, split into sedentary and exercised groups) mdx mice. The EDL and soleus from old mdx mice exhibited decreased absolute twitch and tetanic forces, and the soleus exhibited a >50% decrease in relative forces (13.4 ± 0.4 vs. 6.0 ± 0.9 N/cm2) compared with adult mice. Old mdx muscles also showed longer contraction times and a higher percentage of type I fibers. Normal and mdx mice completed 10 wk of swimming, but mdx mice spent significantly less time swimming than normal animals (7.8 ± 0.4 vs. 15.8 ± 1.1 min, respectively). However, despite their severe dystrophy, mdx muscles responded positively to the low-intensity exercise. Relative tetanic tensions were increased (∼25% and ∼45% for the EDL and soleus, respectively) after the swimming, although absolute forces were unaffected. Thus these results indicate that, even with a dystrophin-deficient myopathy, mdx muscles can still respond to low-intensity exercise. This study shows that the contractile function of muscles of old mdx mice displays many similarities to that of human dystrophic patients and provides further evidence that the use of non-weight-bearing, low-intensity exercises, such as swimming, has no detrimental effect on dystrophic muscle and could be a useful therapeutic aid for sufferers of muscular dystrophy.
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41

Ponzetti, Marco, Argia Ucci, Antonio Maurizi, Luca Giacchi, Anna Teti, and Nadia Rucci. "Lipocalin 2 Influences Bone and Muscle Phenotype in the MDX Mouse Model of Duchenne Muscular Dystrophy." International Journal of Molecular Sciences 23, no. 2 (January 16, 2022): 958. http://dx.doi.org/10.3390/ijms23020958.

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Lipocalin 2 (Lcn2) is an adipokine involved in bone and energy metabolism. Its serum levels correlate with bone mechanical unloading and inflammation, two conditions representing hallmarks of Duchenne Muscular Dystrophy (DMD). Therefore, we investigated the role of Lcn2 in bone loss induced by muscle failure in the MDX mouse model of DMD. We found increased Lcn2 serum levels in MDX mice at 1, 3, 6, and 12 months of age. Consistently, Lcn2 mRNA was higher in MDX versus WT muscles. Immunohistochemistry showed Lcn2 expression in mononuclear cells between muscle fibres and in muscle fibres, thus confirming the gene expression results. We then ablated Lcn2 in MDX mice, breeding them with Lcn2−/− mice (MDXxLcn2−/−), resulting in a higher percentage of trabecular volume/total tissue volume compared to MDX mice, likely due to reduced bone resorption. Moreover, MDXxLcn2−/− mice presented with higher grip strength, increased intact muscle fibres, and reduced serum creatine kinase levels compared to MDX. Consistently, blocking Lcn2 by treating 2-month-old MDX mice with an anti-Lcn2 monoclonal antibody (Lcn2Ab) increased trabecular volume, while reducing osteoclast surface/bone surface compared to MDX mice treated with irrelevant IgG. Grip force was also increased, and diaphragm fibrosis was reduced by the Lcn2Ab. These results suggest that Lcn2 could be a possible therapeutic target to treat DMD-induced bone loss.
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42

Baccari, M. C., S. Nistri, M. G. Vannucchi, F. Calamai, and D. Bani. "Reversal by relaxin of altered ileal spontaneous contractions in dystrophic (mdx) mice through a nitric oxide-mediated mechanism." American Journal of Physiology-Regulatory, Integrative and Comparative Physiology 293, no. 2 (August 2007): R662—R668. http://dx.doi.org/10.1152/ajpregu.00214.2007.

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Altered nitric oxide (NO) production/release is involved in gastrointestinal motor disorders occurring in dystrophic (mdx) mice. Since the hormone relaxin (RLX) can upregulate NO biosynthesis, its effects on spontaneous motility and NO synthase (NOS) expression in the ileum of dystrophic (mdx) mice were investigated. Mechanical responses of ileal preparations were recorded in vitro via force-displacement transducers. Evaluation of the expression of NOS isoforms was performed by immunohistochemistry and Western blot. Normal and mdx mice were distributed into three groups: untreated, RLX pretreated, and vehicle pretreated. Ileal preparations from the untreated animals showed spontaneous muscular contractions whose amplitude was significantly higher in mdx than in normal mice. Addition of RLX, alone or together with l-arginine, to the bath medium depressed the amplitude of the contractions in the mdx mice, thus reestablishing a motility pattern typical of the normal mice. The NOS inhibitor NG-nitro-l-arginine (l-NNA) or the guanylate cyclase inhibitor ODQ reversed the effects of RLX. In RLX-pretreated mdx mice, the amplitude of spontaneous motility was reduced, thus resembling that of the normal mice, and NOS II expression in the muscle coat was increased in respect to the vehicle-pretreated mdx animals. These results indicate that RLX can reverse the altered ileal motility of mdx mice to a normal pattern, likely by upregulating NOS II expression and NO biosynthesis in the ileal smooth muscle.
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43

Tseng, B. S., P. Zhao, J. S. Pattison, S. E. Gordon, J. A. Granchelli, R. W. Madsen, L. C. Folk, E. P. Hoffman, and F. W. Booth. "Regenerated mdx mouse skeletal muscle shows differential mRNA expression." Journal of Applied Physiology 93, no. 2 (August 1, 2002): 537–45. http://dx.doi.org/10.1152/japplphysiol.00202.2002.

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Despite over 3,000 articles published on dystrophin in the last 15 years, the reasons underlying the progression of the human disease, differential muscle involvement, and disparate phenotypes in different species are not understood. The present experiment employed a screen of 12,488 mRNAs in 16-wk-old mouse mdx muscle at a time when the skeletal muscle is avoiding severe dystrophic pathophysiology, despite the absence of a functional dystrophin protein. A number of transcripts whose levels differed between the mdx and human Duchenne muscular dystrophy were noted. A fourfold decrease in myostatin mRNA in the mdx muscle was noted. Differential upregulation of actin-related protein 2/3 (subunit 4), β-thymosin, calponin, mast cell chymase, and guanidinoacetate methyltransferase mRNA in the more benign mdx was also observed. Transcripts for oxidative and glycolytic enzymes in mdx muscle were not downregulated. These discrepancies could provide candidates for salvage pathways that maintain skeletal muscle integrity in the absence of a functional dystrophin protein in mdx skeletal muscle.
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44

Geissinger, H. D., and C. K. McDonald-Taylor. "Fine structure of early degenerating myofibers in the tibialis anterior of young ‘MDX’ mouse." Proceedings, annual meeting, Electron Microscopy Society of America 46 (1988): 322–23. http://dx.doi.org/10.1017/s042482010010367x.

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A new strain of mice, which had arisen by mutation from a dystrophic mouse colony was designated ‘mdx’, because the genetic defect, which manifests itself in brief periods of muscle destruction followed by episodes of muscle regeneration appears to be X-linked. Further studies of histopathological changes in muscle from ‘mdx’ mice at the light microscopic or electron microscopic levels have been published, but only one preliminary study has been on the tibialis anterior (TA) of ‘mdx’ mice less than four weeks old. Lesions in the ‘mdx’ mice vary between different muscles, and centronucleation of fibers in all muscles studied so far appears to be especially prominent in older mice. Lesions in young ‘mdx’ mice have not been studied extensively, and the results appear to be at variance with one another. The degenerative and regenerative aspects of the lesions in the TA of 23 to 26-day-old ‘mdx’ mice appear to vary quantitatively.
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45

Helliwell, T. R., P. A. MacLennan, A. McArdle, R. H. T. Edwards, and M. J. Jackson. "Fasting Increases the Extent of Muscle Necrosis in the mdx Mouse." Clinical Science 90, no. 6 (June 1, 1996): 467–72. http://dx.doi.org/10.1042/cs0900467.

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1. The effects of fasting for 48 h were investigated in CS7BL/10 (wild type) and age-matched C57BL/10 dystrophin-deficient (mdx) mice. 2. Fasting resulted in an increased percentage of necrotic fibres in muscles from the hindlimb and lumbar regions of mdx mice. The percentage of necrotic fibres of forelimb and chest muscles of mdx mice was unaltered by fasting. In wild-type mice, very few necrotic fibres were observed after fasting. 3. The necrotic changes in fasted mdx muscle were not accompanied by altered energy status as evaluated by muscle ATP and phosphocreatine concentrations. 4. A significantly decreased rectal temperature was observed in mdx but not in wild-type mice after fasting. 5. Fasting would normally be expected to cause a reduction in muscle fibre size. The high prevalence of necrosis in fasted mdx mice is therefore an unusual response that may be related to disturbance of the mechanisms which, in the fed state, compensate for the dystrophin deficiency in these animals.
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46

Dempsey, Fiona C., Hussein Al-Ali, Scott J. Crichton, Charlene Fabian, Emily Roberts, Chris Pepper, Michael C. Schmid, and Christopher N. Parris. "Efficacy of MDX-124, a novel anti-annexin-A1 antibody, in preclinical models of pancreatic cancer." Journal of Clinical Oncology 40, no. 4_suppl (February 1, 2022): 590. http://dx.doi.org/10.1200/jco.2022.40.4_suppl.590.

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590 Background: Pancreatic cancer is a highly fatal disease with poor survival and response to both chemotherapy and immunotherapy. Novel approaches to treat this disease are urgently required. Annexin-A1 (ANXA1) is secreted in response to several physiological stimuli where it activates formyl peptide receptors (FPR1/2) triggering multiple oncogenic processes. High ANXA1 expression in pancreatic cancer patients is associated with poor overall survival, and influences cancer progression, drug sensitivity, migration and invasion. MDX-124 is a novel humanized antibody targeting ANXA1 and we have previously presented data demonstrating its significant antiproliferative activity. Here we present further data showing the efficacy of MDX-124 in several preclinical models of pancreatic cancer. Methods: In-vitro models utilized MIA PaCa-2, PANC-1 or BxPC-3 human pancreatic cancer cell lines. Cell cycle progression was evaluated by measuring changes in DNA content via flow cytometry. Pancreatic cancer cell viability following incubation with MDX-124 (0-10 µM) and 5FU (IC50) was assessed via MTT assay. A transwell migration assay was used to evaluate the effect of MDX-124 (0-50 µM) on pancreatic cancer cell migration. In-vivo efficacy was evaluated using an orthotopic mouse model of metastatic pancreatic cancer (FC1242luc/zsGreen; KPC-derived cell line) with bioluminescent imaging used to quantify the incidence and burden of lung metastases. Results: When compared to untreated MIA PaCa-2 pancreatic cancer cells, MDX-124 treatment decreased the proportion of cells in S-phase by 29% and G2 phase by 9.1%, with a concomitant increase in G1 of 38.1%. This occurred in a dose-dependent manner and is consistent with an MDX-124 mediated increase in cell cycle arrest. MDX-124 significantly reduced the viability of MIA PaCa-2 and PANC-1 cell lines versus an IgG control in a dose-dependent manner. Additionally in these two cell lines, combination of MDX-124 with 5FU (IC50) had a significant synergistic impact reducing cancer cell viability by 99.8% and 91.2% respectively. Furthermore, MDX-124 significantly reduced the migratory ability of MIA PaCa-2 and BxPC-3 pancreatic cancer cells. In the orthotopic model of metastatic pancreatic cancer, the murine analog of MDX-124 (MDX-001), markedly reduced both the incidence and size of lung metastases. Conclusions: MDX-124 demonstrated significant anti-tumor efficacy in several preclinical models of pancreatic cancer as a single agent, with increased potency observed when used in combination with 5FU. Medannex will initiate a First-In-Human study in Q4 2021 to evaluate MDX-124 in solid malignancies, including pancreatic cancer.
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47

MacLennan, P. A., A. McArdle, and R. H. Edwards. "Acute effects of phorbol esters on the protein-synthetic rate and carbohydrate metabolism of normal and mdx mouse muscles." Biochemical Journal 275, no. 2 (April 15, 1991): 477–83. http://dx.doi.org/10.1042/bj2750477.

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1. mdx mice do not express dystrophin, the product of the gene which is defective in Duchenne and Becker muscular dystrophy. We have previously shown that protein-synthetic rates (ks) are increased in mdx mouse muscles [MacLennan & Edwards (1990) Biochem. J. 268, 795-797]. 2. The tumour-promoting stereoisomer of phorbol 12,13-didecanoate (4 beta-PDD) acutely increased the ks of muscles from mdx and wild-type (C57BL/10) mice incubated in vitro in the absence of insulin. The effects of 4 beta-PDD are presumably mediated by activation of protein kinase C (PKC). 3. The muscle glycogen concentrations of mdx mice were higher than those of C57BL/10 mice. Studies performed in vivo and in vitro suggested that the effect might be at least partially due to increased rate of glycogen synthesis in mdx muscle. 4. 4 beta-PDD increased the glycogen-synthetic rates rates of C57BL/10, but not mdx, muscles incubated in vitro in the absence of insulin. 5. In muscles from both species incubated in the absence of insulin, treatment with 4 beta-PDD also induced increased rates of glucose uptake and lactate production. Kinetic studies of C57BL/10 and mdx muscles suggested that 4 beta-PDD raised the Vmax. of glucose uptake, but did not alter the Km for the process. 6. The possible role of PKC in controlling the protein and carbohydrate metabolism of normal and mdx mouse muscles is discussed.
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48

Iyer, Shama R., Sameer B. Shah, Ana P. Valencia, Martin F. Schneider, Erick O. Hernández-Ochoa, Joseph P. Stains, Silvia S. Blemker, and Richard M. Lovering. "Altered nuclear dynamics in MDX myofibers." Journal of Applied Physiology 122, no. 3 (March 1, 2017): 470–81. http://dx.doi.org/10.1152/japplphysiol.00857.2016.

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Duchenne muscular dystrophy (DMD) is a genetic disorder in which the absence of dystrophin leads to progressive muscle degeneration and weakness. Although the genetic basis is known, the pathophysiology of dystrophic skeletal muscle remains unclear. We examined nuclear movement in wild-type (WT) and muscular dystrophy mouse model for DMD (MDX) (dystrophin-null) mouse myofibers. We also examined expression of proteins in the linkers of nucleoskeleton and cytoskeleton (LINC) complex, as well as nuclear transcriptional activity via histone H3 acetylation and polyadenylate-binding nuclear protein-1. Because movement of nuclei is not only LINC dependent but also microtubule dependent, we analyzed microtubule density and organization in WT and MDX myofibers, including the application of a unique 3D tool to assess microtubule core structure. Nuclei in MDX myofibers were more mobile than in WT myofibers for both distance traveled and velocity. MDX muscle shows reduced expression and labeling intensity of nesprin-1, a LINC protein that attaches the nucleus to the microtubule and actin cytoskeleton. MDX nuclei also showed altered transcriptional activity. Previous studies established that microtubule structure at the cortex is disrupted in MDX myofibers; our analyses extend these findings by showing that microtubule structure in the core is also disrupted. In addition, we studied malformed MDX myofibers to better understand the role of altered myofiber morphology vs. microtubule architecture in the underlying susceptibility to injury seen in dystrophic muscles. We incorporated morphological and microtubule architectural concepts into a simplified finite element mathematical model of myofiber mechanics, which suggests a greater contribution of myofiber morphology than microtubule structure to muscle biomechanical performance.NEW & NOTEWORTHY Microtubules provide the means for nuclear movement but show altered organization in the muscular dystrophy mouse model (MDX) (dystrophin-null) muscle. Here, MDX myofibers show increased nuclear movement, altered transcriptional activity, and altered linkers of nucleoskeleton and cytoskeleton complex expression compared with healthy myofibers. Microtubule architecture was incorporated in finite element modeling of passive stretch, revealing a role of fiber malformation, commonly found in MDX muscle. The results suggest that alterations in microtubule architecture in MDX muscle affect nuclear movement, which is essential for muscle function.
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49

Mareedu, Satvik, Ronald Pachon, Jayapalraj Thilagavathi, Nadezhda Fefelova, Rekha Balakrishnan, Nandita Niranjan, Lai-Hua Xie, and Gopal J. Babu. "Sarcolipin haploinsufficiency prevents dystrophic cardiomyopathy in mdx mice." American Journal of Physiology-Heart and Circulatory Physiology 320, no. 1 (January 1, 2021): H200—H210. http://dx.doi.org/10.1152/ajpheart.00601.2020.

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First, reducing sarcopolin (SLN) expression improves sarco/endoplasmic reticulum Ca2+ uptake and intracellular Ca2+ handling and prevents cardiomyopathy in mdx mice. Second, reducing SLN expression prevents diastolic dysfunction and improves cardiac contractility in mdx mice Third, reducing SLN expression activates apelin-mediated cardioprotective signaling pathways in mdx heart.
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50

Monceau, Alexandra, Clément Delacroix, Mégane Lemaitre, Gaelle Revet, Denis Furling, Onnik Agbulut, Arnaud Klein, and Arnaud Ferry. "The beneficial effect of chronic muscular exercise on muscle fragility is increased by Prox1 gene transfer in dystrophic mdx muscle." PLOS ONE 17, no. 4 (April 18, 2022): e0254274. http://dx.doi.org/10.1371/journal.pone.0254274.

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Purpose Greater muscle fragility is thought to cause the exhaustion of the muscle stem cells during successive degeneration/repair cycles, leading to muscle wasting and weakness in Duchenne muscular dystrophy. Chronic voluntary exercise can partially reduce the susceptibility to contraction induced-muscle damage, i.e., muscle fragility, as shown by a reduced immediate maximal force drop following lengthening contractions, in the dystrophic mdx mice. Here, we studied the effect of Prospero-related homeobox factor 1 gene (Prox1) transfer (overexpression) using an AAV on fragility in chronically exercised mdx mice, because Prox1 promotes slower type fibres in healthy mice and slower fibres are less fragile in mdx muscle. Methods Both tibialis anterior muscles of the same mdx mouse received the transfer of Prox1 and PBS and the mice performed voluntary running into a wheel during 1 month. We also performed Prox1 transfer in sedentary mdx mice. In situ maximal force production of the muscle in response to nerve stimulation was assessed before, during and after 10 lengthening contractions. Molecular muscle parameters were also evaluated. Results Interestingly, Prox1 transfer reduced the isometric force drop following lengthening contractions in exercised mdx mice (p < 0.05 to 0.01), but not in sedentary mdx mice. It also increased the muscle expression of Myh7 (p < 0.001), MHC-2x (p < 0.01) and Trpc1 (p < 0.01), whereas it reduced that one of Myh4 (p < 0.001) and MHC-2b (p < 0.01) in exercised mdx mice. Moreover, Prox1 transfer decreased the absolute maximal isometric force (p < 0.01), but not the specific maximal isometric force, before lengthening contraction in exercised (p < 0.01) and sedentary mdx mice. Conclusion Our results indicate that Prox1 transfer increased the beneficial effect of chronic exercise on muscle fragility in mdx mice, but reduced absolute maximal force. Thus, the potential clinical benefit of the transfer of Prox1 into exercised dystrophic muscle can merit further investigation.
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