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Artículos de revistas sobre el tema "Muscular dystrophic x-linked (mdx)"

Autor: Grafiati

Publicado: 4 de junio de 2021

Última modificación: 12 de febrero de 2022

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1

Nogami, Ken'ichiro, Yusuke Maruyama, Fusako Sakai-Takemura, Norio Motohashi, Ahmed Elhussieny, Michihiro Imamura, Satoshi Miyashita, et al. "Pharmacological activation of SERCA ameliorates dystrophic phenotypes in dystrophin-deficient mdx mice." Human Molecular Genetics 30, no. 11 (April 5, 2021): 1006–19. http://dx.doi.org/10.1093/hmg/ddab100.

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Abstract Duchenne muscular dystrophy (DMD) is an X-linked genetic disorder characterized by progressive muscular weakness because of the loss of dystrophin. Extracellular Ca2+ flows into the cytoplasm through membrane tears in dystrophin-deficient myofibers, which leads to muscle contracture and necrosis. Sarco/endoplasmic reticulum Ca2+-ATPase (SERCA) takes up cytosolic Ca2+ into the sarcoplasmic reticulum, but its activity is decreased in dystrophic muscle. Here, we show that an allosteric SERCA activator, CDN1163, ameliorates dystrophic phenotypes in dystrophin-deficient mdx mice. The admin

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Carberry, Steven, Margit Zweyer, Dieter Swandulla, and Kay Ohlendieck. "Profiling of Age-Related Changes in theTibialis AnteriorMuscle Proteome of the mdx Mouse Model of Dystrophinopathy." Journal of Biomedicine and Biotechnology 2012 (2012): 1–11. http://dx.doi.org/10.1155/2012/691641.

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X-linked muscular dystrophy is a highly progressive disease of childhood and characterized by primary genetic abnormalities in the dystrophin gene. Senescent mdx specimens were used for a large-scale survey of potential age-related alterations in the dystrophic phenotype, because the established mdx animal model of dystrophinopathy exhibits progressive deterioration of muscle tissue with age. Since the mdxtibialis anteriormuscle is a frequently used model system in muscular dystrophy research, we employed this particular muscle to determine global changes in the dystrophic skeletal muscle prot

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3

Cui, Chang-Hao, Taro Uyama, Kenji Miyado, Masanori Terai, Satoru Kyo, Tohru Kiyono, and Akihiro Umezawa. "Menstrual Blood-derived Cells Confer Human Dystrophin Expression in the Murine Model of Duchenne Muscular Dystrophy via Cell Fusion and Myogenic Transdifferentiation." Molecular Biology of the Cell 18, no. 5 (May 2007): 1586–94. http://dx.doi.org/10.1091/mbc.e06-09-0872.

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Duchenne muscular dystrophy (DMD), the most common lethal genetic disorder in children, is an X-linked recessive muscle disease characterized by the absence of dystrophin at the sarcolemma of muscle fibers. We examined a putative endometrial progenitor obtained from endometrial tissue samples to determine whether these cells repair muscular degeneration in a murine mdx model of DMD. Implanted cells conferred human dystrophin in degenerated muscle of immunodeficient mdx mice. We then examined menstrual blood–derived cells to determine whether primarily cultured nontransformed cells also repair

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Pelosi, Laura, Laura Forcina, Carmine Nicoletti, Bianca Maria Scicchitano, and Antonio Musarò. "Increased Circulating Levels of Interleukin-6 Induce Perturbation in Redox-Regulated Signaling Cascades in Muscle of Dystrophic Mice." Oxidative Medicine and Cellular Longevity 2017 (2017): 1–10. http://dx.doi.org/10.1155/2017/1987218.

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Duchenne muscular dystrophy (DMD) is an X-linked genetic disease in which dystrophin gene is mutated, resulting in dysfunctional or absent dystrophin protein. The pathology of dystrophic muscle includes degeneration, necrosis with inflammatory cell invasion, regeneration, and fibrous and fatty changes. Nevertheless, the mechanisms by which the absence of dystrophin leads to muscle degeneration remain to be fully elucidated. An imbalance between oxidant and antioxidant systems has been proposed as a secondary effect of DMD. However, the significance and precise extent of the perturbation in red

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5

Lewis, Caroline, Harald Jockusch, and Kay Ohlendieck. "Proteomic Profiling of the Dystrophin-Deficient MDX Heart Reveals Drastically Altered Levels of Key Metabolic and Contractile Proteins." Journal of Biomedicine and Biotechnology 2010 (2010): 1–20. http://dx.doi.org/10.1155/2010/648501.

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Although Duchenne muscular dystrophy is primarily classified as a neuromuscular disease, cardiac complications play an important role in the course of this X-linked inherited disorder. The pathobiochemical steps causing a progressive decline in the dystrophic heart are not well understood. We therefore carried out a fluorescence difference in-gel electrophoretic analysis of 9-month-old dystrophin-deficient versus age-matched normal heart, using the established MDX mouse model of muscular dystrophy-related cardiomyopathy. Out of 2,509 detectable protein spots, 79 2D-spots showed a drastic diffe

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Culligan, Kevin, Niamh Banville, Paul Dowling, and Kay Ohlendieck. "Drastic reduction of calsequestrin-like proteins and impaired calcium binding in dystrophic mdx muscle." Journal of Applied Physiology 92, no. 2 (February 1, 2002): 435–45. http://dx.doi.org/10.1152/japplphysiol.00903.2001.

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Although the reduction in dystrophin-associated glycoproteins is the primary pathophysiological consequence of the deficiency in dystrophin, little is known about the secondary abnormalities leading to x-linked muscular dystrophy. As abnormal Ca2+ handling may be involved in myonecrosis, we investigated the fate of key Ca2+ regulatory membrane proteins in dystrophic mdx skeletal muscle membranes. Whereas the expression of the ryanodine receptor, the dihydropyridine receptor, the Ca2+-ATPase, and calsequestrin was not affected, a drastic decline in calsequestrin-like proteins of 150–220 kDa was

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7

Wells, Dominic J., Aurora Ferrer, and Kim E. Wells. "Immunological hurdles in the path to gene therapy for Duchenne muscular dystrophy." Expert Reviews in Molecular Medicine 4, no. 23 (November 4, 2002): 1–23. http://dx.doi.org/10.1017/s146239940200515x.

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Patients with Duchenne muscular dystrophy (DMD), an X-linked lethal muscle-wasting disease, have abnormal expression of the protein dystrophin within their muscle fibres. In the mdx mouse model of this condition, both germline and neonatal somatic gene transfers of dystrophin cDNAs have demonstrated the potential of gene therapy in treating DMD. However, in many DMD patients, there appears to be no dystrophin expression when muscle biopsies are immunostained or western blots are performed. This raises the possibility that the expression of dystrophin following gene transfer might trigger a des

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8

Joseph, Josiane, Dong Cho, and Jason Doles. "Metabolomic Analyses Reveal Extensive Progenitor Cell Deficiencies in a Mouse Model of Duchenne Muscular Dystrophy." Metabolites 8, no. 4 (October 3, 2018): 61. http://dx.doi.org/10.3390/metabo8040061.

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Duchenne muscular dystrophy (DMD) is a musculoskeletal disorder that causes severe morbidity and reduced lifespan. Individuals with DMD have an X-linked mutation that impairs their ability to produce functional dystrophin protein in muscle. No cure exists for this disease and the few therapies that are available do not dramatically delay disease progression. Thus, there is a need to better understand the mechanisms underlying DMD which may ultimately lead to improved treatment options. The muscular dystrophy (MDX) mouse model is frequently used to explore DMD disease traits. Though some studie

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9

Schertzer, Jonathan D., Chris van der Poel, Thea Shavlakadze, Miranda D. Grounds, and Gordon S. Lynch. "Muscle-specific overexpression of IGF-I improves E-C coupling in skeletal muscle fibers from dystrophic mdx mice." American Journal of Physiology-Cell Physiology 294, no. 1 (January 2008): C161—C168. http://dx.doi.org/10.1152/ajpcell.00399.2007.

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Duchenne muscular dystrophy (DMD) is a lethal X-linked disease caused by the absence of functional dystrophin. Abnormal excitation-contraction (E-C) coupling has been reported in dystrophic muscle fibers from mdx mice, and alterations in E-C coupling components may occur as a direct result of dystrophin deficiency. We hypothesized that muscle-specific overexpression of insulin-growth factor-1 (IGF-I) would reduce E-C coupling failure in mdx muscle. Mechanically skinned extensor digitorum longus muscle fibers from mdx mice displayed a faster decline in depolarization-induced force responses (DI

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10

Petrof, B. J., H. H. Stedman, J. B. Shrager, J. Eby, H. L. Sweeney, and A. M. Kelly. "Adaptations in myosin heavy chain expression and contractile function in dystrophic mouse diaphragm." American Journal of Physiology-Cell Physiology 265, no. 3 (September 1, 1993): C834—C841. http://dx.doi.org/10.1152/ajpcell.1993.265.3.c834.

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The X chromosome-linked muscular dystrophic (mdx) mouse lacks the subsarcolemmal protein dystrophin and thus represents a genetic homologue of human Duchenne muscular dystrophy. The present study examined alterations in diaphragm contractile properties and myosin heavy chain (MHC) expression in young (3-4 mo) and old (22-24 mo) control and mdx mice. In young mdx mice, maximum isometric tension (Po) was reduced to 50% of control values. An increase in fibers coexpressing types I (slow) and IIa MHC as well as regenerating fibers expressing embryonic MHC occurred, whereas IIx/b fibers were decrea

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DOWLING, Paul, Philip DORAN, and Kay OHLENDIECK. "Drastic reduction of sarcalumenin in Dp427 (dystrophin of 427 kDa)-deficient fibres indicates that abnormal calcium handling plays a key role in muscular dystrophy." Biochemical Journal 379, no. 2 (April 15, 2004): 479–88. http://dx.doi.org/10.1042/bj20031311.

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Although the primary abnormality in dystrophin is the underlying cause for mdx (X-chromosome-linked muscular dystrophy), abnormal Ca2+ handling after sarcolemmal microrupturing appears to be the pathophysiological mechanism leading to muscle weakness. To develop novel pharmacological strategies for eliminating Ca2+-dependent proteolysis, it is crucial to determine the fate of Ca2+-handling proteins in dystrophin-deficient fibres. In the present study, we show that a key luminal Ca2+-binding protein SAR (sarcalumenin) is affected in mdx skeletal-muscle fibres. One- and two-dimensional immunoblo

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12

Dangain, J., and IR Neering. "Mouse Models of Muscular Dystrophy: Gene Products and Function." Physiology 7, no. 5 (October 1, 1992): 195–99. http://dx.doi.org/10.1152/physiologyonline.1992.7.5.195.

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With the discovery of the X-linked gene product dystrophin, the mdx mouse came to be regarded as the only suitable mouse model of human muscular dystrophy. However, existence of an autosomal gene homologous with dystrophin, together with physiological evidence of membrane fragility, reestablishes autosomal mouse mutants (dy, dy2j) as valid models.

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13

Bujold, Mathieu, Nicolas Caron, Goeffrey Camiran, Santwana Mukherjee, Paul D. Allen, Jacques P. Tremblay, and Yaming Wang. "Autotransplantation in mdx Mice of mdx Myoblasts Genetically Corrected by an HSV-1 Amplicon Vector." Cell Transplantation 11, no. 8 (November 2002): 759–67. http://dx.doi.org/10.3727/000000002783985297.

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Duchenne muscular dystrophy (DMD) is an X-linked recessive disorder, characterized by a lack of dystrophin. To eliminate the need for immunosuppressive drugs, transplantation of genetically modified autologous myoblasts has been proposed as a possible therapy for this myopathy. An HSV-1 amplicon vector (HSVDGN), containing a 17.3-kb full-length MCK-driven mouse dystrophin cDNA, an eGFP gene, and a neomycin resistance gene driven by CMV or SV40 promoters, respectively, was constructed and used to transduce mdx primary myoblasts. The presence of the eGFP and neomycin resistance genes facilitated

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14

McARDLE, Anne, Timothy R. HELLIWELL, Geoffrey J. BECKETT, Mariana CATAPANO, Anthony DAVIS, and Malcolm J. JACKSON. "Effect of propylthiouracil-induced hypothyroidism on the onset of skeletal muscle necrosis in dystrophin-deficient mdx mice." Clinical Science 95, no. 1 (July 1, 1998): 83–89. http://dx.doi.org/10.1042/cs0950083.

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1.Duchenne and Becker muscular dystrophies are X-linked disorders caused by defects in muscle dystrophin. The mdx mouse is an animal model for Duchenne muscular dystrophy which has a point mutation in the dystrophin gene, resulting in little (< 3%) or no expression of dystrophin in muscle. Mdx mice show a characteristic pattern of muscle necrosis and regeneration. Muscles are normal until the third postnatal week when widespread necrosis commences. This is followed by muscle regeneration, with the persistence of centrally nucleated fibres. 2.This work has examined the hypothesis that the on

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15

Kwak, Dongmin, Guoxian Wei, LaDora V. Thompson, and Jong-Hee Kim. "Short-Term ONX-0914 Administration: Performance and Muscle Phenotype in Mdx Mice." International Journal of Environmental Research and Public Health 17, no. 14 (July 19, 2020): 5211. http://dx.doi.org/10.3390/ijerph17145211.

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Duchenne muscular dystrophy (DMD) is a severe muscle-wasting disease. Although the lack of dystrophin protein is the primary defect responsible for the development of DMD, secondary disease complications such as persistent inflammation contribute greatly to the pathogenesis and the time-dependent progression of muscle destruction. The immunoproteasome is a potential therapeutic target for conditions or diseases mechanistically linked to inflammation. In this study, we explored the possible effects of ONX-0914 administration, an inhibitor specific for the immunoproteasome subunit LMP7 (ß5i), on

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Chan, S., S. I. Head, and J. W. Morley. "Branched fibers in dystrophic mdx muscle are associated with a loss of force following lengthening contractions." American Journal of Physiology-Cell Physiology 293, no. 3 (September 2007): C985—C992. http://dx.doi.org/10.1152/ajpcell.00128.2007.

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We demonstrated that the susceptibility of skeletal muscle to injury from lengthening contractions in the dystrophin-deficient mdx mouse is directly linked with the extent of fiber branching within the muscles and that both parameters increase as the mdx animal ages. We subjected isolated extensor digitorum longus muscles to a lengthening contraction protocol of 15% strain and measured the resulting drop in force production (force deficit). We also examined the morphology of individual muscle fibers. In mdx mice 1–2 mo of age, 17% of muscle fibers were branched, and the force deficit of 7% was

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Meyers, Tatyana A., Jackie A. Heitzman, and DeWayne Townsend. "DMD carrier model with mosaic dystrophin expression in the heart reveals complex vulnerability to myocardial injury." Human Molecular Genetics 29, no. 6 (January 24, 2020): 944–54. http://dx.doi.org/10.1093/hmg/ddaa015.

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Abstract Duchenne muscular dystrophy (DMD) is a devastating neuromuscular disease that causes progressive muscle wasting and cardiomyopathy. This X-linked disease results from mutations of the DMD allele on the X-chromosome resulting in the loss of expression of the protein dystrophin. Dystrophin loss causes cellular dysfunction that drives the loss of healthy skeletal muscle and cardiomyocytes. As gene therapy strategies strive toward dystrophin restoration through micro-dystrophin delivery or exon skipping, preclinical models have shown that incomplete restoration in the heart results in het

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Menke, A., and H. Jockusch. "Extent of shock-induced membrane leakage in human and mouse myotubes depends on dystrophin." Journal of Cell Science 108, no. 2 (February 1, 1995): 727–33. http://dx.doi.org/10.1242/jcs.108.2.727.

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A lack of the cytoskeletal protein dystrophin causes muscle fiber necrosis in Duchenne/Becker muscular dystrophies (DMD/BMD) and in murine X-linked muscular dystrophy (MDX). However, no overt disease symptoms are observed in dystrophin-less cultured myotubes, and the biological function of dystrophin in normal muscle cells is still unknown. In this work, we have extended our studies on a model system, using hypoosmotic shock to determine stress resistance of muscle cells. In frozen sections of control human and mouse myotubes, dystrophin was shown to be localized at the cell periphery as in ma

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Gibson, A. J., J. Karasinski, J. Relvas, J. Moss, T. G. Sherratt, P. N. Strong, and D. J. Watt. "Dermal fibroblasts convert to a myogenic lineage in mdx mouse muscle." Journal of Cell Science 108, no. 1 (January 1, 1995): 207–14. http://dx.doi.org/10.1242/jcs.108.1.207.

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Duchenne muscular dystrophy is a primary muscle disease that manifests itself in young boys as a result of a defect in a gene located on the X-chromosome. This gene codes for dystrophin, a normal muscle protein that is located beneath the sarcolemma of muscle fibres. Therapies to alleviate this disease have centred on implanting normal muscle precursor cells into dystrophic fibres to compensate for the lack of this gene and its product. To date, donor cells for implantation in such therapy have been of myogenic origin, derived from paternal biopsies. Success in human muscle, however, has been

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Dudley, Roy W. R., Maya Khairallah, Shawn Mohammed, Larry Lands, Christine Des Rosiers, and Basil J. Petrof. "Dynamic responses of the glutathione system to acute oxidative stress in dystrophic mouse (mdx) muscles." American Journal of Physiology-Regulatory, Integrative and Comparative Physiology 291, no. 3 (September 2006): R704—R710. http://dx.doi.org/10.1152/ajpregu.00031.2006.

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The precise mechanisms underlying skeletal muscle damage in Duchenne muscular dystrophy (DMD) remain ill-defined. Functional ischemia during muscle activation, with subsequent reperfusion during rest, has been documented. Therefore, one possibility is the presence of increased oxidative stress. We applied a model of acute hindlimb ischemia/reperfusion (I/R) in mdx mice (genetic homolog of DMD) to evaluate dynamic in vivo responses of dystrophic muscles to this form of oxidative stress. Before the application of I/R, mdx muscles showed: 1) decreased levels of total glutathione (GSH) with an inc

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Yanay, Nurit, Moran Elbaz, Jenya Konikov-Rozenman, Sharona Elgavish, Yuval Nevo, Yakov Fellig, Malcolm Rabie, Stella Mitrani-Rosenbaum, and Yoram Nevo. "Pax7, Pax3 and Mamstr genes are involved in skeletal muscle impaired regeneration of dy2J/dy2J mouse model of Lama2-CMD." Human Molecular Genetics 28, no. 20 (July 26, 2019): 3369–90. http://dx.doi.org/10.1093/hmg/ddz180.

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Abstract Congenital muscular dystrophy type-1A (Lama2-CMD) and Duchenne muscular dystrophy (DMD) result from deficiencies of laminin-α2 and dystrophin proteins, respectively. Although both proteins strengthen the sarcolemma, they are implicated in clinically distinct phenotypes. We used RNA-deep sequencing (RNA-Seq) of dy2J/dy2J, Lama2-CMD mouse model, skeletal muscle at 8 weeks of age to elucidate disease pathophysiology. This study is the first report of dy2J/dy2J model whole transcriptome profile. RNA-Seq of the mdx mouse model of DMD and wild-type (WT) mouse was carried as well in order to

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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 subtract

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Tanabe, Y., K. Esaki, and T. Nomura. "Skeletal muscle pathology in X chromosome-linked muscular dystrophy (mdx) mouse." Acta Neuropathologica 69, no. 1-2 (1986): 91–95. http://dx.doi.org/10.1007/bf00687043.

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Kurihara, Teruyuki, Masahiko Kishi, Nobuyuki Saito, Michiji Komoto, Takanobu Hidaka, and Masao Kinoshita. "Electrical myotonia and cataract in X-linked muscular dystrophy (mdx) mouse." Journal of the Neurological Sciences 99, no. 1 (October 1990): 83–92. http://dx.doi.org/10.1016/0022-510x(90)90202-x.

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Laws, Nicola, and Andrew Hoey. "Progression of kyphosis in mdx mice." Journal of Applied Physiology 97, no. 5 (November 2004): 1970–77. http://dx.doi.org/10.1152/japplphysiol.01357.2003.

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Spinal deformity in the form of kyphosis or kyphoscoliosis occurs in most patients with Duchenne muscular dystrophy (DMD), a fatal X-linked disorder caused by an absence of the subsarcolemmal protein dystrophin. Mdx mice, which also lack dystrophin, show thoracolumbar kyphosis that progresses with age. We hypothesize that paraspinal and respiratory muscle weakness and fibrosis are associated with the progression of spinal deformity in this mouse model, and similar to DMD patients there is evidence of altered thoracic conformation and area. We measured kyphosis in mdx and age-matched control mi

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Hui, Tiankun, Hongyang Jing, Tian Zhou, Peng Chen, Ziyang Liu, Xia Dong, Min Yan, et al. "Increasing LRP4 diminishes neuromuscular deficits in a mouse model of Duchenne muscular dystrophy." Human Molecular Genetics 30, no. 17 (May 13, 2021): 1579–90. http://dx.doi.org/10.1093/hmg/ddab135.

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Abstract Duchenne muscular dystrophy (DMD) is an X-linked neuromuscular disease characterized by progressive wasting of skeletal muscles. The neuromuscular junction (NMJ) is a synapse between motor neurons and skeletal muscle fibers, critical for the control of muscle contraction. The NMJ decline is observed in DMD patients, but the mechanism is unclear. LRP4 serves as a receptor for agrin, a proteoglycan secreted by motor neurons to induce NMJ, and plays a critical role in NMJ formation and maintenance. Interestingly, we found that protein levels of LRP4 were reduced both in muscles of the DM

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Barton, Elisabeth R., Linda Morris, Antonio Musaro, Nadia Rosenthal, and H. Lee Sweeney. "Muscle-specific expression of insulin-like growth factor I counters muscle decline in mdx mice." Journal of Cell Biology 157, no. 1 (April 1, 2002): 137–48. http://dx.doi.org/10.1083/jcb.200108071.

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Duchenne muscular dystrophy is an X-linked degenerative disorder of muscle caused by the absence of the protein dystrophin. A major consequence of muscular dystrophy is that the normal regenerative capacity of skeletal muscle cannot compensate for increased susceptibility to damage, leading to repetitive cycles of degeneration–regeneration and ultimately resulting in the replacement of muscle fibers with fibrotic tissue. Because insulin-like growth factor I (IGF-I) has been shown to enhance muscle regeneration and protein synthetic pathways, we asked whether high levels of muscle-specific expr

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Chao, D. S., J. R. Gorospe, J. E. Brenman, J. A. Rafael, M. F. Peters, S. C. Froehner, E. P. Hoffman, J. S. Chamberlain, and D. S. Bredt. "Selective loss of sarcolemmal nitric oxide synthase in Becker muscular dystrophy." Journal of Experimental Medicine 184, no. 2 (August 1, 1996): 609–18. http://dx.doi.org/10.1084/jem.184.2.609.

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Becker muscular dystrophy is an X-linked disease due to mutations of the dystrophin gene. We now show that neuronal-type nitric oxide synthase (nNOS), an identified enzyme in the dystrophin complex, is uniquely absent from skeletal muscle plasma membrane in many human Becker patients and in mouse models of dystrophinopathy. An NH2-terminal domain of nNOS directly interacts with alpha 1-syntrophin but not with other proteins in the dystrophin complex analyzed. However, nNOS does not associate with alpha 1-syntrophin on the sarcolemma in transgenic mdx mice expressing truncated dystrophin protei

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Geissinge, H. D., and L. D. Rhodes. "Regenerating myofibers in tibialis anterior muscles of young ‘MDX’ mice." Proceedings, annual meeting, Electron Microscopy Society of America 45 (August 1987): 726–27. http://dx.doi.org/10.1017/s0424820100127943.

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A recently discovered mouse model (‘mdx’) for muscular dystrophy in man may be of considerable interest, since the disease in ‘mdx’ mice is inherited by the same mode of inheritance (X-linked) as the human Duchenne (DMD) muscular dystrophy. Unlike DMD, which results in a situation in which the continual muscle destruction cannot keep up with abortive regenerative attempts of the musculature, and the sufferers of the disease die early, the disease in ‘mdx’ mice appears to be transient, and the mice do not die as a result of it. In fact, it has been reported that the severely damaged Tibialis an

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Al-Mshhdani, Basma A., Miranda D. Grounds, Peter G. Arthur, and Jessica R. Terrill. "A Blood Biomarker for Duchenne Muscular Dystrophy Shows That Oxidation State of Albumin Correlates with Protein Oxidation and Damage in Mdx Muscle." Antioxidants 10, no. 8 (August 3, 2021): 1241. http://dx.doi.org/10.3390/antiox10081241.

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Duchenne muscular dystrophy (DMD) is a severe X-linked muscle wasting disease with no cure. While the precise mechanisms of progressive dystropathology remain unclear, oxidative stress caused by excessive generation of oxidants is strongly implicated. Blood biomarkers that could track oxidant levels in tissues would be valuable to measure the effectiveness of clinical treatments for DMD; our research has focused on developing such biomarkers. One target of oxidants that has the potential to be harnessed as a clinical biomarker is the thiol side chain of cysteine 34 (Cys34) of the blood protein

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Rae, Mark G., and Dervla O'Malley. "Cognitive dysfunction in Duchenne muscular dystrophy: a possible role for neuromodulatory immune molecules." Journal of Neurophysiology 116, no. 3 (September 1, 2016): 1304–15. http://dx.doi.org/10.1152/jn.00248.2016.

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Duchenne muscular dystrophy (DMD) is an X chromosome-linked disease characterized by progressive physical disability, immobility, and premature death in affected boys. Underlying the devastating symptoms of DMD is the loss of dystrophin, a structural protein that connects the extracellular matrix to the cell cytoskeleton and provides protection against contraction-induced damage in muscle cells, leading to chronic peripheral inflammation. However, dystrophin is also expressed in neurons within specific brain regions, including the hippocampus, a structure associated with learning and memory fo

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Nguyen, T. M., J. M. Ellis, D. R. Love, K. E. Davies, K. C. Gatter, G. Dickson, and G. E. Morris. "Localization of the DMDL gene-encoded dystrophin-related protein using a panel of nineteen monoclonal antibodies: presence at neuromuscular junctions, in the sarcolemma of dystrophic skeletal muscle, in vascular and other smooth muscles, and in proliferating brain cell lines." Journal of Cell Biology 115, no. 6 (December 15, 1991): 1695–700. http://dx.doi.org/10.1083/jcb.115.6.1695.

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mAbs have been raised against different epitopes on the protein product of the DMDL gene, which is an autosomal homologue of the X-linked DMD gene for dystrophin. These antibodies provide direct evidence that DMDL protein is localized near acetylcholine receptors at neuromuscular junctions in normal and mdx mouse intercostal muscle. The primary location in tissues other than skeletal muscle is smooth muscle, especially in the vascular system, which may account for the wide tissue distribution previously demonstrated by Western blotting. The DMDL protein was undetectable in the nonjunctional sa

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Viola, Helena M., Stefan M. K. Davies, Aleksandra Filipovska, and Livia C. Hool. "L-type Ca2+ channel contributes to alterations in mitochondrial calcium handling in the mdx ventricular myocyte." American Journal of Physiology-Heart and Circulatory Physiology 304, no. 6 (March 15, 2013): H767—H775. http://dx.doi.org/10.1152/ajpheart.00700.2012.

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The L-type Ca2+ channel is the main route for calcium entry into cardiac myocytes, and it is essential for contraction. Alterations in whole cell L-type Ca2+ channel current and Ca2+ homeostasis have been implicated in the development of cardiomyopathies. Cytoskeletal proteins can influence whole cell L-type Ca2+ current and mitochondrial function. Duchenne muscular dystrophy is a fatal X-linked disease that leads to progressive muscle weakness due to the absence of cytoskeletal protein dystrophin. This includes dilated cardiomyopathy, but the mechanisms are not well understood. We sought to i

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Siemionow, Maria, M. Malik, P. Langa, J. Cwykiel, S. Brodowska, and A. Heydemann. "Cardiac Protection after Systemic Transplant of Dystrophin Expressing Chimeric (DEC) Cells to the mdx Mouse Model of Duchenne Muscular Dystrophy." Stem Cell Reviews and Reports 15, no. 6 (October 15, 2019): 827–41. http://dx.doi.org/10.1007/s12015-019-09916-0.

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Abstract Duchenne Muscular Dystrophy (DMD) is a progressive lethal disease caused by X-linked mutations of the dystrophin gene. Dystrophin deficiency clinically manifests as skeletal and cardiac muscle weakness, leading to muscle wasting and premature death due to cardiac and respiratory failure. Currently, no cure exists. Since heart disease is becoming a leading cause of death in DMD patients, there is an urgent need to develop new more effective therapeutic strategies for protection and improvement of cardiac function. We previously reported functional improvements correlating with dystroph

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Dell'Agnola, Chiara, Zejing Wang, Rainer Storb, Stephen J. Tapscott, Christian S. Kuhr, Stephen D. Hauschka, Richard S. Lee, et al. "Hematopoietic stem cell transplantation does not restore dystrophin expression in Duchenne muscular dystrophy dogs." Blood 104, no. 13 (December 15, 2004): 4311–18. http://dx.doi.org/10.1182/blood-2004-06-2247.

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Abstract Duchenne muscular dystrophy (DMD) is caused by mutations in the dystrophin gene on the X-chromosome that result in skeletal and cardiac muscle damage and premature death. Studies in mice, including the mdx mouse model of DMD, have demonstrated that circulating bone marrow–derived cells can participate in skeletal muscle regeneration, but the potential clinical utility of treating human DMD by allogeneic marrow transplantation from a healthy donor remains unknown. To assess whether allogeneic hematopoietic cell transplantation (HCT) provides clinically relevant levels of donor muscle c

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Pasternak, C., S. Wong, and E. L. Elson. "Mechanical function of dystrophin in muscle cells." Journal of Cell Biology 128, no. 3 (February 1, 1995): 355–61. http://dx.doi.org/10.1083/jcb.128.3.355.

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We have directly measured the contribution of dystrophin to the cortical stiffness of living muscle cells and have demonstrated that lack of dystrophin causes a substantial reduction in stiffness. The inferred molecular structure of dystrophin, its preferential localization underlying the cell surface, and the apparent fragility of muscle cells which lack this protein suggest that dystrophin stabilizes the sarcolemma and protects the myofiber from disruption during contraction. Lacking dystrophin, the muscle cells of persons with Duchenne muscular dystrophy (DMD) are abnormally vulnerable. The

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Kress, W., T. Grimm, C. R. Müller, H. Keller, and R. Bittner. "Parental Source Effect on Inherited Mutations in the Dystrophin Gene of Mice and Humans." Acta geneticae medicae et gemellologiae: twin research 45, no. 1-2 (April 1996): 251–53. http://dx.doi.org/10.1017/s0001566000001409.

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Skewed X inactivation has been suspected as the genetic cause for some female carriers of Duchenne/Becker muscular dystrophy (DMD/BMD) presenting symptoms [1], as well as in manifesting females in other X-linked recessive diseases. No clear “parental source effect” – a difference in phenotype depending upon transmission of the mutated allele by either the father or the mother – has been observed [Cremer]. To date, most studies in this field have analysed the methylation status of flanking polymorphic sites (a measure of the maintenance of X inactivation and imprinting), not the expression of t

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Shibuya, S., and Y. Wakayama. "Freeze-fracture studies of myofiber plasma membrane in X chromosome-linked muscular dystrophy (mdx) mice." Acta Neuropathologica 76, no. 2 (1988): 179–84. http://dx.doi.org/10.1007/bf00688102.

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Lessa, Thais Borges, Rafael Cardoso Carvalho, Júlio David Spagnolo, Luis Claudio Lopes Correia da Silva, Silvia Renata Gaido Cortopassi, and Carlos Eduardo Ambrósio. "Laparoscopic guided local injection in the X-linked muscular dystrophy mouse (mdx) diaphragm. An advance in experimental therapies for Duchenne Muscular Dystrophy." Acta Cirurgica Brasileira 29, no. 11 (November 2014): 715–20. http://dx.doi.org/10.1590/s0102-86502014001800004.

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Dowling, Paul, Margit Zweyer, Maren Raucamp, Michael Henry, Paula Meleady, Dieter Swandulla, and Kay Ohlendieck. "Dataset on the mass spectrometry-based proteomic profiling of the kidney from wild type and the dystrophic mdx-4cv mouse model of X-linked muscular dystrophy." Data in Brief 28 (February 2020): 105067. http://dx.doi.org/10.1016/j.dib.2019.105067.

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Klymiuk, N., C. Thirion, K. Burkhardt, A. Wuensch, S. Krause, A. Richter, B. Kessler, et al. "238 TAILORED PIG MODEL OF DUCHENNE MUSCULAR DYSTROPHY." Reproduction, Fertility and Development 24, no. 1 (2012): 231. http://dx.doi.org/10.1071/rdv24n1ab238.

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Duchenne muscular dystrophy (DMD) is one of the most common genetic diseases in humans, affecting 1 in 3500 boys. It is characterised by progressive muscle weakness and wasting due to mutations in the dystrophin (DMD) gene resulting in absence of dystrophin protein in skeletal muscle. Although curative treatments are currently not available, genetic and pharmacological approaches are under investigation including early-phase clinical trials. Existing animal models in different species (e.g. mdx mouse, GRMD dog) have been instrumental to understand the pathophysiology of DMD, but have several l

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Watt, Diana J., Janusz Karasinski, and Marjorie A. England. "Migration of lacZ positive cells from the tibialis anterior to the extensor digitorum longus muscle of the X-linked muscular dystrophic (mdx) mouse." Journal of Muscle Research and Cell Motility 14, no. 1 (February 1993): 121–32. http://dx.doi.org/10.1007/bf00132186.

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Sano, M., Y. Wada, K. Ii, E. Kominami, N. Katunuma, and H. Tsukagoshi. "Immunolocalization of cathepsins B, H and L in skeletal muscle of X-linked muscular dystrophy (mdx) mouse." Acta Neuropathologica 75, no. 3 (1988): 217–25. http://dx.doi.org/10.1007/bf00690529.

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Xin, Can, Xiangyu Chu, Wenzhong Wei, Biao Kuang, Yiqing Wang, Ying Tang, Jincao Chen, Hongbo You, Chengwen Li, and Bing Wang. "Combined gene therapy via VEGF and mini-dystrophin synergistically improves pathologies in temporalis muscle of dystrophin/utrophin double knockout mice." Human Molecular Genetics 30, no. 14 (May 13, 2021): 1349–59. http://dx.doi.org/10.1093/hmg/ddab120.

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Abstract Duchenne muscular dystrophy (DMD) is a severe X-linked inherited muscular disorder characterized by the loss of dystrophin. We have previously shown that monogene therapy using the mini-dystrophin gene improves muscle function in DMD. However, chronic inflammation plays an important role in progressive muscle degeneration in DMD as well. Vascular endothelial growth factor (VEGF) has been used to enhance muscle vasculature, reduce local inflammation and improve DMD muscle function. Temporalis muscles are the key skeletal muscles for mastication and loss of their function negatively aff

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Fukada, So-ichiro, Yuko Miyagoe-Suzuki, Hiroshi Tsukihara, Katsutoshi Yuasa, Saito Higuchi, Shiro Ono, Kazutake Tsujikawa, Shin'ichi Takeda, and Hiroshi Yamamoto. "Muscle regeneration by reconstitution with bone marrow or fetal liver cells from green fluorescent protein-gene transgenic mice." Journal of Cell Science 115, no. 6 (March 15, 2002): 1285–93. http://dx.doi.org/10.1242/jcs.115.6.1285.

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The myogenic potential of bone marrow and fetal liver cells was examined using donor cells from green fluorescent protein (GFP)-gene transgenic mice transferred into chimeric mice. Lethally irradiated X-chromosome-linked muscular dystrophy (mdx) mice receiving bone marrow cells from the transgenic mice exhibited significant numbers of fluorescence+ and dystrophin+ muscle fibres. In order to compare the generating capacity of fetal liver cells with bone marrow cells in neonatal chimeras,these two cell types from the transgenic mice were injected into busulfantreated normal or mdx neonatal mice,

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Lilling, Gilla, and Rivka Beitner. "Altered allosteric properties of cytoskeleton-bound phosphofructokinase in muscle from mice with X chromosome-linked muscular dystrophy (mdx)." Biochemical Medicine and Metabolic Biology 45, no. 3 (June 1991): 319–25. http://dx.doi.org/10.1016/0885-4505(91)90036-k.

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Frinchi, Monica, Giuseppe Morici, Giuseppa Mudó, Maria Bonsignore, and Valentina Di Liberto. "Beneficial Role of Exercise in the Modulation of mdx Muscle Plastic Remodeling and Oxidative Stress." Antioxidants 10, no. 4 (April 3, 2021): 558. http://dx.doi.org/10.3390/antiox10040558.

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Duchenne muscular dystrophy (DMD) is an X-linked recessive progressive lethal disorder caused by the lack of dystrophin, which determines myofibers mechanical instability, oxidative stress, inflammation, and susceptibility to contraction-induced injuries. Unfortunately, at present, there is no efficient therapy for DMD. Beyond several promising gene- and stem cells-based strategies under investigation, physical activity may represent a valid noninvasive therapeutic approach to slow down the progression of the pathology. However, ethical issues, the limited number of studies in humans and the l

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Barraza-Flores, Pamela, Tatiana M. Fontelonga, Ryan D. Wuebbles, Hailey J. Hermann, Andreia M. Nunes, Joe N. Kornegay, and Dean J. Burkin. "Laminin-111 protein therapy enhances muscle regeneration and repair in the GRMD dog model of Duchenne muscular dystrophy." Human Molecular Genetics 28, no. 16 (April 24, 2019): 2686–95. http://dx.doi.org/10.1093/hmg/ddz086.

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Abstract Duchenne muscular dystrophy (DMD) is a devastating X-linked disease affecting ~1 in 5000 males. DMD patients exhibit progressive muscle degeneration and weakness, leading to loss of ambulation and premature death from cardiopulmonary failure. We previously reported that mouse Laminin-111 (msLam-111) protein could reduce muscle pathology and improve muscle function in the mdx mouse model for DMD. In this study, we examined the ability of msLam-111 to prevent muscle disease progression in the golden retriever muscular dystrophy (GRMD) dog model of DMD. The msLam-111 protein was injected

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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-

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Hirn, Carole, George Shapovalov, Olivier Petermann, Emmanuelle Roulet, and Urs T. Ruegg. "Nav1.4 Deregulation in Dystrophic Skeletal Muscle Leads to Na+ Overload and Enhanced Cell Death." Journal of General Physiology 132, no. 2 (July 14, 2008): 199–208. http://dx.doi.org/10.1085/jgp.200810024.

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Duchenne muscular dystrophy (DMD) is a hereditary degenerative disease manifested by the absence of dystrophin, a structural, cytoskeletal protein, leading to muscle degeneration and early death through respiratory and cardiac muscle failure. Whereas the rise of cytosolic Ca2+ concentrations in muscles of mdx mouse, an animal model of DMD, has been extensively documented, little is known about the mechanisms causing alterations in Na+ concentrations. Here we show that the skeletal muscle isoform of the voltage-gated sodium channel, Nav1.4, which represents over 90% of voltage-gated sodium chan

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