Relevant bibliographies by topics / D2-mdx

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Academic literature on the topic 'D2-mdx'

Author: Grafiati
Published: 25 May 2024

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Journal articles on the topic "D2-mdx"

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De Giorgio, Daria, Deborah Novelli, Francesca Motta, Marianna Cerrato, Davide Olivari, Annasimon Salama, Francesca Fumagalli, et al. "Characterization of the Cardiac Structure and Function of Conscious D2.B10-Dmdmdx/J (D2-mdx) mice from 16–17 to 24–25 Weeks of Age." International Journal of Molecular Sciences 24, no. 14 (July 22, 2023): 11805. http://dx.doi.org/10.3390/ijms241411805.

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Duchenne muscular dystrophy (DMD) is the most common form of muscle degenerative hereditary disease. Muscular replacement by fibrosis and calcification are the principal causes of progressive and severe musculoskeletal, respiratory, and cardiac dysfunction. To date, the D2.B10-Dmdmdx/J (D2-mdx) model is proposed as the closest to DMD, but the results are controversial. In this study, the cardiac structure and function was characterized in D2-mdx mice from 16–17 up to 24–25 weeks of age. Echocardiographic assessment in conscious mice, gross pathology, and histological and cardiac biomarker analyses were performed. At 16–17 weeks of age, D2-mdx mice presented mild left ventricular function impairment and increased pulmonary vascular resistance. Cardiac fibrosis was more extended in the right ventricle, principally on the epicardium. In 24–25-week-old D2-mdx mice, functional and structural alterations increased but with large individual variation. High-sensitivity cardiac Troponin T, but not N-terminal pro-atrial natriuretic peptide, plasma levels were increased. In conclusion, left ventricle remodeling was mild to moderate in both young and adult mice. We confirmed that right ventricle epicardial fibrosis is the most outstanding finding in D2-mdx mice. Further long-term studies are needed to evaluate whether this mouse model can also be considered a model of DMD cardiomyopathy.
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Hassani, Medhi, Dylan Moutachi, Mégane Lemaitre, Alexis Boulinguiez, Denis Furling, Onnik Agbulut, and Arnaud Ferry. "Beneficial effects of resistance training on both mild and severe mouse dystrophic muscle function as a preclinical option for Duchenne muscular dystrophy." PLOS ONE 19, no. 3 (March 8, 2024): e0295700. http://dx.doi.org/10.1371/journal.pone.0295700.

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Mechanical overloading (OVL) resulting from the ablation of muscle agonists, a supra-physiological model of resistance training, reduces skeletal muscle fragility, i.e. the immediate maximal force drop following lengthening contractions, and increases maximal force production, in mdx mice, a murine model of Duchene muscular dystrophy (DMD). Here, we further analyzed these beneficial effects of OVL by determining whether they were blocked by cyclosporin, an inhibitor of the calcineurin pathway, and whether there were also observed in the D2-mdx mice, a more severe murine DMD model. We found that cyclosporin did not block the beneficial effect of 1-month OVL on plantaris muscle fragility in mdx mice, nor did it limit the increases in maximal force and muscle weight (an index of hypertrophy). Fragility and maximal force were also ameliorated by OVL in the plantaris muscle of D2-mdx mice. In addition, OVL increased the expression of utrophin, cytoplamic γ-actin, MyoD, and p-Akt in the D2-mdx mice, proteins playing an important role in fragility, maximal force gain and muscle growth. In conclusion, OVL reduced fragility and increased maximal force in the more frequently used mild mdx model but also in D2-mdx mice, a severe model of DMD, closer to human physiopathology. Moreover, these beneficial effects of OVL did not seem to be related to the activation of the calcineurin pathway. Thus, this preclinical study suggests that resistance training could have a potential benefit in the improvement of the quality of life of DMD patients.
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Hayes, Holly M., Julie Angerosa, Adam T. Piers, Jason D. White, Jane Koleff, Madeline Thurgood, Jessica Moody, Michael M. Cheung, and Salvatore Pepe. "Preserved Left Ventricular Function despite Myocardial Fibrosis and Myopathy in the Dystrophin-Deficient D2.B10-Dmdmdx/J Mouse." Oxidative Medicine and Cellular Longevity 2022 (March 16, 2022): 1–19. http://dx.doi.org/10.1155/2022/5362115.

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Duchenne muscular dystrophy involves an absence of dystrophin, a cytoskeletal protein which supports cell structural integrity and scaffolding for signalling molecules in myocytes. Affected individuals experience progressive muscle degeneration that leads to irreversible loss of ambulation and respiratory diaphragm function. Although clinical management has greatly advanced, heart failure due to myocardial cell loss and fibrosis remains the major cause of death. We examined cardiac morphology and function in D2.B10-Dmdmdx/J (D2-mdx) mice, a relatively new mouse model of muscular dystrophy, which we compared to their wild-type background DBA/2J mice (DBA/2). We also tested whether drug treatment with a specific blocker of mitochondrial permeability transition pore opening (Debio-025), or ACE inhibition (Perindopril), had any effect on dystrophy-related cardiomyopathy. D2-mdx mice were treated for six weeks with Vehicle control, Debio-025 (20 mg/kg/day), Perindopril (2 mg/kg/day), or a combination ( n = 8 /group). At 18 weeks, compared to DBA/2, D2-mdx hearts displayed greater ventricular collagen, lower cell density, greater cell diameter, and greater protein expression levels of IL-6, TLR4, BAX/Bcl2, caspase-3, PGC-1α, and notably monoamine oxidases A and B. Remarkably, these adaptations in D2-mdx mice were associated with preserved resting left ventricular function similar to DBA/2 mice. Compared to vehicle, although Perindopril partly attenuated the increase in heart weight and collagen at 18 weeks, the drug treatments had no marked impact on dystrophic cardiomyopathy.
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Krishna, Swathy, Tiffany Quindry, Matthew B. Hudson, John C. Quindry, and Joshua T. Selsby. "Defective Autophagic Degradation in Aged D2‐mdx Diaphragms." FASEB Journal 34, S1 (April 2020): 1. http://dx.doi.org/10.1096/fasebj.2020.34.s1.04955.

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5

Yarlagadda, Sai, Christina Kulis, Peter G. Noakes, and Mark L. Smythe. "Hematopoietic Prostaglandin D Synthase Inhibitor PK007 Decreases Muscle Necrosis in DMD mdx Model Mice." Life 11, no. 9 (September 21, 2021): 994. http://dx.doi.org/10.3390/life11090994.

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Duchenne muscular dystrophy (DMD) is characterized by progressive muscle weakness and wasting due to the lack of dystrophin protein. The acute phase of DMD is characterized by muscle necrosis and increased levels of the pro-inflammatory mediator, prostaglandin D2 (PGD2). Inhibiting the production of PGD2 by inhibiting hematopoietic prostaglandin D synthase (HPGDS) may alleviate inflammation and decrease muscle necrosis. We tested our novel HPGDS inhibitor, PK007, in the mdx mouse model of DMD. Our results show that hindlimb grip strength was two-fold greater in the PK007-treated mdx group, compared to untreated mdx mice, and displayed similar muscle strength to strain control mice (C57BL/10ScSn). Histological analyses showed a decreased percentage of regenerating muscle fibers (~20% less) in tibialis anterior (TA) and gastrocnemius muscles and reduced fibrosis in the TA muscle in PK007-treated mice. Lastly, we confirmed that the DMD blood biomarker, muscle creatine kinase activity, was also reduced by ~50% in PK007-treated mdx mice. We conclude that our HPGDS inhibitor, PK007, has effectively reduced muscle inflammation and fibrosis in a DMD mdx mouse model.
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Spaulding, Hannah R., Tiffany Quindry, Kayleen Hammer, John C. Quindry, and Joshua T. Selsby. "Nutraceutical and pharmaceutical cocktails did not improve muscle function or reduce histological damage in D2-mdx mice." Journal of Applied Physiology 127, no. 4 (October 1, 2019): 1058–66. http://dx.doi.org/10.1152/japplphysiol.00162.2019.

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Progressive muscle injury and weakness are hallmarks of Duchenne muscular dystrophy. We showed previously that quercetin (Q) partially protected dystrophic limb muscles from disease-related injury. As quercetin activates PGC-1α through Sirtuin-1, an NAD+-dependent deacetylase, the depleted NAD+ in dystrophic skeletal muscle may limit quercetin efficacy; hence, supplementation with the NAD+ donor, nicotinamide riboside (NR), may facilitate quercetin efficacy. Lisinopril (Lis) protects skeletal muscle and improves cardiac function in dystrophin-deficient mice; therefore, it was included in this study to evaluate the effects of lisinopril used with quercetin and NR. Our purpose was to determine the extent to which Q, NR, and Lis decreased dystrophic injury. We hypothesized that Q, NR, or Lis alone would improve muscle function and decrease histological injury and when used in combination would have additive effects. Muscle function of 11-mo-old DBA (healthy), D2-mdx (dystrophin-deficient), and D2-mdx mice was assessed after treatment with Q, NR, and/or Lis for 7 mo. To mimic typical pharmacology of patients with Duchenne muscular dystrophy, a group was treated with prednisolone (Pred) in combination with Q, NR, and Lis. At 11 mo of age, dystrophin deficiency decreased specific tension and tetanic force in the soleus and extensor digitorum longus muscles and was not corrected by any treatment. Dystrophic muscle was more sensitive to contraction-induced injury, which was partially offset in the QNRLisPred group, whereas fatigue was similar between all groups. Treatments did not decrease histological damage. These data suggest that treatment with Q, NR, Lis, and Pred failed to adequately maintain dystrophic limb muscle function or decrease histological damage. NEW & NOTEWORTHY Despite a compelling rationale and previous evidence to the contrary in short-term investigations, quercetin, nicotinamide riboside, or Lisinopril, alone or in combination, failed to restore muscle function or decrease histological injury in dystrophic limb muscle from D2-mdx mice after long-term administration. Importantly, we also found that in the D2-mdx model, an emerging and relatively understudied model of Duchenne muscular dystrophy dystrophin deficiency caused profound muscle dysfunction and histopathology in skeletal muscle.
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Martins-Bach, A., E. Araujo, B. Matot, Y. Fromes, P. Baudin, I. Richard, and P. Carlier. "Nuclear magnetic resonance relaxometry characterization of D2-mdx mice." Neuromuscular Disorders 27 (October 2017): S124. http://dx.doi.org/10.1016/j.nmd.2017.06.120.

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8

Ward, Christopher W., Frederick Sachs, Ernest D. Bush, and Thomas M. Suchyna. "GsMTx4-D provides protection to the D2.mdx mouse." Neuromuscular Disorders 28, no. 10 (October 2018): 868–77. http://dx.doi.org/10.1016/j.nmd.2018.07.005.

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9

Pandeya, Sarbesh R., Janice A. Nagy, Daniela Riveros, Carson Semple, Rebecca S. Taylor, Benjamin Sanchez, and Seward B. Rutkove. "Relationships between in vivo surface and ex vivo electrical impedance myography measurements in three different neuromuscular disorder mouse models." PLOS ONE 16, no. 10 (October 29, 2021): e0259071. http://dx.doi.org/10.1371/journal.pone.0259071.

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Electrical impedance myography (EIM) using surface techniques has shown promise as a means of diagnosing and tracking disorders affecting muscle and assessing treatment efficacy. However, the relationship between such surface-obtained impedance values and pure muscle impedance values has not been established. Here we studied three groups of diseased and wild-type (WT) animals, including a Duchenne muscular dystrophy model (the D2-mdx mouse), an amyotrophic lateral sclerosis (ALS) model (the SOD1 G93A mouse), and a model of fat-related atrophy (the db/db diabetic obese mouse), performing hind limb measurements using a standard surface array and ex vivo measurements on freshly excised gastrocnemius muscle. A total of 101 animals (23 D2-mdx, 43 ALS mice, 12 db/db mice, and corresponding 30 WT mice) were studied with EIM across a frequency range of 8 kHz to 1 MHz. For both D2-mdx and ALS models, moderate strength correlations (Spearman rho values generally ranging from 0.3–0.7, depending on the impedance parameter (i.e., resistance, reactance and phase) were obtained. In these groups of animals, there was an offset in frequency with impedance values obtained at higher surface frequencies correlating more strongly to impedance values obtained at lower ex vivo frequencies. For the db/db model, correlations were comparatively weaker and strongest at very high and very low frequencies. When combining impedance data from all three disease models together, moderate correlations persisted (with maximal Spearman rho values of 0.45). These data support that surface EIM data reflect ex vivo muscle tissue EIM values to a moderate degree across several different diseases, with the highest correlations occurring in the 10–200 kHz frequency range. Understanding these relationships will prove useful for future applications of the technique of EIM in the assessment of neuromuscular disorders.
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English, Katherine G., Andrea L. Reid, Adrienne Samani, Gerald J. F. Coulis, S. Armando Villalta, Christopher J. Walker, Sharon Tamir, and Matthew S. Alexander. "Next-Generation SINE Compound KPT−8602 Ameliorates Dystrophic Pathology in Zebrafish and Mouse Models of DMD." Biomedicines 10, no. 10 (September 26, 2022): 2400. http://dx.doi.org/10.3390/biomedicines10102400.

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Duchenne muscular dystrophy (DMD) is a progressive, X-linked childhood neuromuscular disorder that results from loss-of-function mutations in the DYSTROPHIN gene. DMD patients exhibit muscle necrosis, cardiomyopathy, respiratory failure, and loss of ambulation. One of the major driving forces of DMD disease pathology is chronic inflammation. The current DMD standard of care is corticosteroids; however, there are serious side effects with long-term use, thus identifying novel anti-inflammatory and anti-fibrotic treatments for DMD is of high priority. We investigated the next-generation SINE compound, KPT−8602 (eltanexor) as an oral therapeutic to alleviate dystrophic symptoms. We performed pre-clinical evaluation of the effects of KPT−8602 in DMD zebrafish (sapje) and mouse (D2-mdx) models. KPT−8602 improved dystrophic skeletal muscle pathologies, muscle architecture and integrity, and overall outcomes in both animal models. KPT−8602 treatment ameliorated DMD pathology in D2-mdx mice, with increased locomotor behavior and improved muscle histology. KPT−8602 altered the immunological profile of the dystrophic mice, and reduced circulating osteopontin serum levels. These findings demonstrate KPT−8602 as an effective therapeutic in DMD through by promotion of an anti-inflammatory environment and overall improvement of DMD pathological outcomes.
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Dissertations / Theses on the topic "D2-mdx"

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Monceau, Alexandra. "Effet de l'exercice physique, combiné ou non à une thérapie génique, sur la fonction musculaire de modèles murins de dystrophie musculaire de Duchenne." Electronic Thesis or Diss., Sorbonne université, 2021. https://accesdistant.sorbonne-universite.fr/login?url=https://theses-intra.sorbonne-universite.fr/2021SORUS172.pdf.

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La dystrophie musculaire de Duchenne (DMD) est une maladie neuromusculaire causée par des mutations du gène DMD codant la dystrophine. Son absence engendre une fragilité musculaire accrue, et une faiblesse musculaire extrême. Il n’existe pas encore de traitement curatif, mais l’utilisation de plusieurs approches thérapeutiques combinées semble prometteuse. Nous avons testé l’idée que l’activité physique régulière pourrait être un moyen de diminuer les symptômes dystrophiques du muscle squelettique, dans des modèles murins de DMD. Tout d’abord, nous avons évalué l’effet d’un exercice physique chronique d’endurance lorsqu'il est associé à la surexpression de Prox1, un facteur de transcription connu pour favoriser des fibres plus lentes dans un muscle sain, celles-ci étant moins atteintes dans la DMD. Nous avons mis en évidence que cette combinaison permettait de diminuer la fragilité musculaire, chez la souris mdx, le modèle murin classique de la DMD, et avait donc le potentiel d’arrêter la progression de la pathologie. Par la suite, nous avons regardé l’effet de l’exercice physique chronique d’endurance lorsqu’il est combiné à une thérapie génique, qui restaure l’expression de la dystrophine, chez la souris D2-mdx, un modèle sévère de DMD. Nous avons montré que l’exercice chronique d’endurance diminuait l’efficacité de la thérapie génique, en diminuant la restauration de la dystrophine. Enfin, nous avons caractérisé les effets de l’exercice physique chronique de résistance chez la souris D2-mdx. Nos résultats indiquent une amélioration très importante de la fonction musculaire en réponse à la surcharge mécanique, sans dommages musculaires évidents dans ce modèle sévère
Duchenne muscular dystrophy (DMD) is a neuromuscular disease caused by mutations in the DMD gene encoding dystrophin, a protein essential for the integrity of the skeletal muscle fiber. Its absence causes increased muscle fragility, and extreme muscle weakness. Currently, there is no curative treatment, but the use of several combined therapeutic approaches seems promising. We tested the idea that regular physical activity could be a way to decrease dystrophic symptoms of skeletal muscle, especially those related to muscle function, in mice models of DMD. First, we evaluated the effect of chronic endurance exercise when combined with overexpression of Prox1, a transcription factor known to promote slower fibers in healthy muscle, which are less affected in DMD. We demonstrated that this combination allowed to decrease muscle fragility in mdx mice, the classical mouse model of DMD, and thus had the potential to stop the progression of the disease. Subsequently, we were interested in the effect of chronic endurance exercise when combined with gene therapy, which restores dystrophin expression, in D2-mdx mouse, a severe model of DMD. We showed that chronic endurance exercise decreased the efficiency of the gene therapy, by decreasing the restoration of dystrophin. Finally, we characterized the effects of chronic resistance exercise in D2-mdx mice. Our results indicate an incredibly significant improvement in muscle function in response to mechanical overload, without obvious muscle damage in this severe model
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2

(11191884), Bohyun Ro. "IMPACT OF HEAT THERAPY ON SKELETAL MUSCLE FUNCTION IN A MODEL OF DUCHENNE MUSCULAR DYSTROPHY." Thesis, 2021.

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Current study demonstrated the impact of heat therapy on skeletal muscle function in a model of Duchenne muscular dystrophy (DMD). The aim of this study was to: (1) examine the impact of treatment temperature on the skeletal muscle adaptation in DBA/2J mice; and (2) determine the impact of repeated HT for 3 consecutive weeks on body composition and skeletal muscle function in D2.mdx, a model of DMD. From study 1, we revealed that HT at 39℃ for 3 weeks significantly promoted relative muscle mass of both EDL and soleus muscle in DBA/2J mice. However, from study 2, HT at 39℃ for 3 weeks does not improve muscle function or increase muscle mass in a mouse model of DMD.
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Book chapters on the topic "D2-mdx"

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Kennedy, Tahnee L., and Hannah F. Dugdale. "Cardiac and Skeletal Muscle Pathology in the D2/mdx Mouse Model and Caveats Associated with the Quantification of Utrophin." In Methods in Molecular Biology, 55–66. New York, NY: Springer US, 2022. http://dx.doi.org/10.1007/978-1-0716-2772-3_4.

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Kennedy, Tahnee L., and Hannah F. Dugdale. "Correction to: Cardiac and Skeletal Muscle Pathology in the D2/mdx Mouse Model and Caveats Associated with the Quantification of Utrophin." In Methods in Molecular Biology, C1. New York, NY: Springer US, 2023. http://dx.doi.org/10.1007/978-1-0716-2772-3_31.

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