☆ 4.4 Article

Exercise and duchenne muscular dystrophy: Where we have been and where we need to go

MUSCLE & NERVE (2012)

Journal

MUSCLE & NERVE

Volume 45, Issue 5, Pages 746-751

Publisher

WILEY

DOI: 10.1002/mus.23244

Keywords

DMD; dystrophic; mdx; mechanisms; muscle; physical activity

Categories

Clinical Neurology Neurosciences

Ask authors/readers for more resources

Protocol

Community support

Reagent

Community support

Authors

I am an author on this paper

Click your name to claim this paper and add it to your profile.

Reviews

Primary Rating

4.4

Not enough ratings

Secondary Ratings

Novelty

-

Significance

-

Scientific rigor

-

Rate this paper

Recommended

Article Biochemistry & Molecular Biology

Pregabalin synchronizes the regeneration of nerve and muscle fibers optimizing the gait recovery of MDX dystrophic mice

Alex Dias Assis, Gabriela Bortolanca Chiarotto, Natalia Scanavachia da Silva, Gustavo Ferreira Simoes, Alexandre Leite Rodrigues Oliveira

Summary: The study demonstrates that pregabalin can reduce the retrograde impact of muscle degeneration/regeneration on the nervous system in Duchenne muscular dystrophy (DMD) and enhance axonal regeneration, leading to improved motor function. The treatment shows no side effects and upregulates the expression of pro-regenerative proteins in neurons and Schwann cells.

FASEB JOURNAL (2022)

Add to Collection

Article Neurosciences

Pregabalin-induced neuroprotection and gait improvement in dystrophic MDX mice

Alex Dias Assis, Gabriela Bortolanca Chiarotto, Gustavo Ferreira Simoes, Alexandre Leite Rodrigues Oliveira

Summary: Treatment with pregabalin (PGB) can protect peripheral nerves in MDX mice, improve motor coordination, and potentially be translated to clinical applications in the future.

MOLECULAR AND CELLULAR NEUROSCIENCE (2021)

Add to Collection

Article Physiology

Indicators of increased ER stress and UPR in aged D2-mdx and human dystrophic skeletal muscles

Swathy Krishna, Hannah R. R. Spaulding, James E. E. Koltes, John C. C. Quindry, Rudy J. J. Valentine, Joshua T. T. Selsby

Summary: Duchenne muscular dystrophy (DMD) is a progressive muscle disease that results in muscle wasting, wheelchair dependence, and eventual death. This study aimed to understand the alterations of ER stress and the UPR in dystrophic muscles. The results showed upregulation of ER stress markers and the UPR in D2-mdx mice and human dystrophic muscles. Transcription factors that regulate these changes were also identified.

FRONTIERS IN PHYSIOLOGY (2023)

Add to Collection

Article Biology

Accelerating the Mdx Heart Histo-Pathology through Physical Exercise

Jacopo Morroni, Leonardo Schirone, Daniele Vecchio, Carmine Nicoletti, Luca D'Ambrosio, Valentina Valenti, Sebastiano Sciarretta, Biliana Lozanoska-Ochser, Marina Bouche

Summary: Chronic cardiac muscle inflammation and fibrosis are key features of Duchenne Muscular Dystrophy. The widely used DMD animal model, the mdx mouse, shows milder cardiac pathology compared to humans. A study demonstrated that exercise can accelerate and worsen the cardiac pathology in mdx mice, providing a useful tool for studying dystrophic heart alterations and evaluating therapeutic strategies.

LIFE-BASEL (2021)

Add to Collection

Article Physiology

Lifespan Analysis of Dystrophic mdx Fast-Twitch Muscle Morphology and Its Impact on Contractile Function

Leonit Kiriaev, Sindy Kueh, John W. Morley, Kathryn N. North, Peter J. Houweling, Stewart I. Head

Summary: This study on Duchenne muscular dystrophy in mice found that older mice experienced significant loss of muscle strength after eccentric contractions, with an increase in branched fiber complexity. These findings have important implications for pre-clinical drug studies and genetic rescue strategies.

FRONTIERS IN PHYSIOLOGY (2021)

Add to Collection

Article Physiology

Lipidomic Analyses Reveal Specific Alterations of Phosphatidylcholine in Dystrophic Mdx Muscle

William J. Valentine, Sherif A. Mostafa, Suzumi M. Tokuoka, Fumie Hamano, Natsuko F. Inagaki, Joel Z. Nordin, Norio Motohashi, Yoshihiro Kita, Yoshitsugu Aoki, Takao Shimizu, Hideo Shindou

Summary: In Duchenne muscular dystrophy (DMD), changes in phosphatidylcholine (PC) levels, specifically higher levels of PC 34:1 and lower levels of PC 34:2, are associated with muscle wasting. The study found that PC 34:1 levels were elevated in regenerated mdx muscles, while PC 34:2 levels were also elevated in mdx muscles. Experimental factors such as muscle types, mouse ages, and diets were found to impact the PC alterations.

FRONTIERS IN PHYSIOLOGY (2022)

Add to Collection

Article Cell Biology

Electrophysiological analysis of healthy and dystrophic 3-D bioengineered skeletal muscle tissues

Christine T. Nguyen, Majid Ebrahimi, Penney M. Gilbert, Bryan A. Stewart

Summary: This study analyzed the electrophysiological characteristics of healthy and diseased three-dimensional bioengineered skeletal muscle tissues, focusing on Duchenne muscular dystrophy. The research also tested a potential therapeutic compound and showed improvement in membrane potential in dystrophic muscle, laying the foundation for examining potential therapies for muscular disorders in vitro.

AMERICAN JOURNAL OF PHYSIOLOGY-CELL PHYSIOLOGY (2021)

Add to Collection

Article Geriatrics & Gerontology

RhoA/ROCK signalling activated by ARHGEF3 promotes muscle weakness via autophagy in dystrophic mdx mice

Jae-Sung You, Yongdeok Kim, Soohyun Lee, Rashid Bashir, Jie Chen

Summary: The RhoA/ROCK signaling pathway plays a critical role in the pathology of Duchenne muscular dystrophy (DMD), and inhibiting ROCK can improve muscle strength and quality in DMD by rescuing autophagy.

JOURNAL OF CACHEXIA SARCOPENIA AND MUSCLE (2023)

Add to Collection

Article Biochemistry & Molecular Biology

Proteome Profiling of the Dystrophic mdx Mice Diaphragm

Olga Mucha, Malgorzata Myszka, Paulina Podkalicka, Bianka Swiderska, Agata Malinowska, Jozef Dulak, Agnieszka Loboda

Summary: This study investigated differentially expressed proteins in the diaphragm of 6-week-old mdx mice, a model for Duchenne muscular dystrophy (DMD), using label-free and tandem mass tag (TMT)-based methods. The comparison of both methods revealed 88 commonly changed proteins. Further analysis showed that 953 proteins significantly changed in dystrophic animals, with 867 increased and 86 decreased in expression. Interestingly, a significant decrease in the expression of enzymes generating hydrogen sulfide (H2S) was found in the dystrophic diaphragm, suggesting a potential role of H2S metabolism in modulating DMD progression.

BIOMOLECULES (2023)

Add to Collection

Article Cell Biology

The location of protein oxidation in dystrophic skeletal muscle from the mdx mouse model of Duchenne muscular dystrophy

Tomohito Iwasaki, Jessica R. Terrill, Kei Kawarai, Yusei Miyata, Takayoshi Tagami, Naoyuki Maeda, Yasuhiro Hasegawa, Takafumi Watanabe, Miranda D. Grounds, Peter G. Arthur

Summary: This study visualised the precise location of different oxidative modifications to proteins in dystrophic muscles using the dystrophic mdx mouse model for Duchenne muscular dystrophy (DMD). The results showed a correlation between irreversible protein oxidation, specifically dityrosine formation, and areas of myonecrosis. Measuring protein oxidation, particularly dityrosines, may be a useful biomarker for indirectly quantifying myonecrosis in research studies using mdx mice and other animal models for DMD.

ACTA HISTOCHEMICA (2022)

Add to Collection

Article Medicine, Research & Experimental

CD38-NADase is a new major contributor to Duchenne muscular dystrophic phenotype

Antoine Zelicourt, Abdallah Fayssoil, Mbarka Dakouane-Giudicelli, Isley De Jesus, Ahmed Karoui, Faouzi Zarrouki, Florence Lefebvre, Arnaud Mansart, Jean-Marie Launay, Jerome Piquereau, Mariana G. Tarrago, Marcel Bonay, Anne Forand, Sophie Moog, France Pietri-Rouxel, Elise Brisebard, Claudia C. S. Chini, Sonu Kashyap, Matthew J. Fogarty, Gary C. Sieck, Mathias Mericskay, Eduardo N. Chini, Ana Maria Gomez, Jose-Manuel Cancela, Sabine Porte

Summary: This study demonstrates that CD38 plays an active role in the pathophysiology of Duchenne muscular dystrophy (DMD). Deletion of CD38 in mdx mice leads to restored heart function and structure, improved skeletal muscle performance, reduced inflammation and senescence markers, and restored muscle NAD(+) levels. Treatment with CD38 inhibitors also improves skeletal muscle performance in DMD mice. These findings suggest that selective anti-CD38 therapeutic intervention could be a promising approach for treating DMD patients.

EMBO MOLECULAR MEDICINE (2022)

Add to Collection

Article Cardiac & Cardiovascular Systems

Sarcolipin haploinsufficiency prevents dystrophic cardiomyopathy in mdx mice

Satvik Mareedu, Ronald Pachon, Jayapalraj Thilagavathi, Nadezhda Fefelova, Rekha Balakrishnan, Nandita Niranjan, Lai-Hua Xie, Gopal J. Babu

Summary: Reducing SLN expression improves cardiac SERCA function and Ca(2+)i cycling, prevents cardiomyopathy in mdx mice, activates apelin-mediated cardioprotective signaling pathways, and enhances cardiac function.

AMERICAN JOURNAL OF PHYSIOLOGY-HEART AND CIRCULATORY PHYSIOLOGY (2021)

Add to Collection

Article Physiology

Circadian Genes as Exploratory Biomarkers in DMD: Results From Both the mdx Mouse Model and Patients

Rachele Rossi, Maria Sofia Falzarano, Hana Osman, Annarita Armaroli, Chiara Scotton, Paola Mantuano, Brigida Boccanegra, Ornella Cappellari, Elena Schwartz, Anton Yuryev, Eugenio Mercuri, Enrico Bertini, Adele D'Amico, Marina Mora, Camilla Johansson, Cristina Al-Khalili Szigyarto, Annamaria De Luca, Alessandra Ferlini

Summary: Duchenne muscular dystrophy (DMD) is a rare genetic disease causing progressive weakness and muscle wasting due to dystrophin gene mutations. Circadian rhythm plays a key role in maintaining muscle functions and the study identified CSNK1E, SIRT1, and MYOG as potential circadian biomarkers in DMD patients.

FRONTIERS IN PHYSIOLOGY (2021)

Add to Collection

Article Physiology

Six weeks of N-acetylcysteine antioxidant in drinking water decreases pathological fiber branching in MDX mouse dystrophic fast-twitch skeletal muscle

Asma Redwan, Leonit Kiriaev, Sindy Kueh, John W. Morley, Peter Houweling, Ben D. Perry, Stewart I. Head

Summary: This study tested the hypothesis that adding the antioxidant NAC to the drinking water of mdx mice could treat the inflammatory phase and reduce pathological muscle fiber branching and splitting. The results showed that NAC treatment significantly reduced muscle mass and abnormal fiber branching and splitting in mdx EDL muscles.

FRONTIERS IN PHYSIOLOGY (2023)

Add to Collection

Article Biochemistry & Molecular Biology

Inhibition of PKCθ Improves Dystrophic Heart Phenotype and Function in a Novel Model of DMD Cardiomyopathy

Jacopo Morroni, Leonardo Schirone, Valentina Valenti, Clemens Zwergel, Carles Sanchez Riera, Sergio Valente, Daniele Vecchio, Sonia Schiavon, Rino Ragno, Antonello Mai, Sebastiano Sciarretta, Biliana Lozanoska-Ochser, Marina Bouche

Summary: Chronic cardiac muscle inflammation and subsequent fibrotic tissue deposition are key features in Duchenne Muscular Dystrophy (DMD). Corticosteroids are currently the treatment of choice for delaying DMD progression, but there is a need for new anti-inflammatory therapies due to the adverse effects associated with long-term corticosteroid use. In this study, the inhibition of PKC theta was found to significantly reduce immune cell infiltration, necrosis, and fibrosis in the heart. Functional improvements were also observed in left ventricle fractional shortening. These findings suggest that PKC theta pharmacological inhibition could be an attractive therapeutic approach for treating dystrophic cardiomyopathy.

INTERNATIONAL JOURNAL OF MOLECULAR SCIENCES (2022)

Add to Collection

Review Clinical Neurology

Diagnosis and management of Duchenne muscular dystrophy, part 2: respiratory, cardiac, bone health, and orthopaedic management

David J. Birnkrant, Katharine Bushby, Carla M. Bann, Benjamin A. Alman, Susan D. Apkon, Angela Blackwell, Laura E. Case, Linda Cripe, Stasia Hadjiyannakis, Aaron K. Olson, Daniel W. Sheehan, Julie Bolen, David R. Weber, Leanne M. Ward

LANCET NEUROLOGY (2018)

Add to Collection

Article Biotechnology & Applied Microbiology

Correction of Biochemical Abnormalities and Improved Muscle Function in a Phase I/II Clinical Trial of Clenbuterol in Pompe Disease

Dwight D. Koeberl, Laura E. Case, Edward C. Smith, Crista Walters, Sang-oh Han, Yanzhen Li, Wei Chen, Christoph P. Hornik, Kim M. Huffman, William E. Kraus, Beth L. Thurberg, David L. Corcoran, Deeksha Bali, Nenad Bursac, Priya S. Kishnani

MOLECULAR THERAPY (2018)

Add to Collection

Article Endocrinology & Metabolism

Early-onset of symptoms and clinical course of Pompe disease associated with the c.-32-13 T > G variant

Mrudu Herbert, Laura E. Case, Mugdha Rairikar, Heidi Cope, Lauren Bailey, Stephanie L. Austin, Priya S. Kishnani

MOLECULAR GENETICS AND METABOLISM (2019)

Add to Collection

Editorial Material Pediatrics

Commentary on Progression of Ankle Plantarflexion Contractures and Functional Decline in Duchenne Muscular Dystrophy: Implications for Physical Therapy Management

Laura E. Case, Julie Coats

PEDIATRIC PHYSICAL THERAPY (2019)

Add to Collection

Review Pediatrics

Physical Activity Levels of Children With Down Syndrome

Bianca Fox, Gwendolyn E. Moffett, Clara Kinnison, Grace Brooks, Laura E. Case

PEDIATRIC PHYSICAL THERAPY (2019)

Add to Collection

Article Cardiac & Cardiovascular Systems

Cardioprotective effects of idebenone do not involve ROS scavenging: Evidence for mitochondrial complex I bypass in ischemia/reperfusion injury

Justin B. Perry, Grace N. Davis, Mitchell E. Allen, Marina Makrecka-Kuka, Maija Dambrova, Robert W. Grange, Saame Raza Shaikh, David A. Brown

JOURNAL OF MOLECULAR AND CELLULAR CARDIOLOGY (2019)

Add to Collection

Article Biotechnology & Applied Microbiology

AAV-Mediated Gene Transfer Restores a Normal Muscle Transcriptome in a Canine Model of X-Linked Myotubular Myopathy

Jean-Baptiste Dupont, Jianjun Guo, Edith Renaud-Gabardos, Karine Poulard, Virginie Latournerie, Michael W. Lawlor, Robert W. Grange, John T. Gray, Ana Buj-Bello, Martin K. Childers, David L. Mack

MOLECULAR THERAPY (2020)

Add to Collection

Review Physiology

Pro-opiomelanocortin Neurons and the Transcriptional Regulation of Motivated Exercise

Deborah J. Good, Haiyan Zhang, Robert W. Grange, Thomas Braun

EXERCISE AND SPORT SCIENCES REVIEWS (2020)

Add to Collection

Article Cell Biology

Potential cross-talk between muscle and tendon in Duchenne muscular dystrophy

Claire Yuan, Ashwin Arora, Anthony M. Garofalo, Robert W. Grange

Summary: This study explores the potential signaling between dystrophic skeletal muscle and tendon in Duchenne muscular dystrophy, focusing on the cross-talk at the myotendinous junction. The absence of dystrophin and the associated dystrophin-glycoprotein complex is a key feature of Duchenne muscular dystrophy, with other potential signal pathways contributing to the cross-talk between muscle and tendon.

CONNECTIVE TISSUE RESEARCH (2021)

Add to Collection

Article Physiology

A novel ex vivo protocol to mimic human walking gait: implications for Duchenne muscular dystrophy

Katherine E. Bukovec, Xiao Hu, Matthew Borkowski, Duane Jeffery, Silvia S. Blemker, Robert W. Grange

JOURNAL OF APPLIED PHYSIOLOGY (2020)

Add to Collection

Article Physiology

A novel murine muscle loading model to investigate Achilles musculotendinous adaptation

Sabah N. Rezvani, Anne E. C. Nichols, Robert W. Grange, Linda A. Dahlgren, P. Gunnar Brolinson, Vincent M. Wang

Summary: Achilles tendinopathy is a challenging condition with limited effective therapies. This study introduced a novel mouse model of hindlimb muscle loading for targeted therapeutic exercise, showing promising results in improving biomechanical outcomes in a murine tendinopathy model. This model opens up possibilities for further research on how muscle loading can enhance healing of Achilles tendon injuries.

JOURNAL OF APPLIED PHYSIOLOGY (2021)

Add to Collection

Article Medicine, Research & Experimental

Voluntary wheel running complements microdystrophin gene therapy to improve muscle function in mdx mice

Shelby E. Hamm, Daniel D. Fathalikhani, Katherine E. Bukovec, Adele K. Addington, Haiyan Zhang, Justin B. Perry, Ryan P. McMillan, Michael W. Lawlor, Mariah J. Prom, Mark A. Vanden Avond, Suresh N. Kumar, Kirsten E. Coleman, J. B. Dupont, David L. Mack, David A. Brown, Carl A. Morris, J. Patrick Gonzalez, Robert W. Grange

Summary: The study showed that combining voluntary wheel running with microdystrophin gene therapy in young mdx mice improved whole-body performance, affected muscle function to varying degrees, mitigated energy deficits, but also revealed some detrimental effects of exercise.

MOLECULAR THERAPY-METHODS & CLINICAL DEVELOPMENT (2021)

Add to Collection

Article Nutrition & Dietetics

Dietary Conjugated Linoleic Acid Reduces Body Weight and Fat in Snord116m+/p- and Snord116m-/p- Mouse Models of Prader-Willi Syndrome

Brittney Knott, Matthew A. Kocher, Henry A. Paz, Shelby E. Hamm, William Fink, Jordan Mason, Robert W. Grange, Umesh D. Wankhade, Deborah J. Good

Summary: This study demonstrated that Snord116(m+/p-) mice and mice with a deletion of both Snord116 alleles showed weight and fat loss on a high-fat/CLA diet, indicating that the genotype did not interfere with CLA actions. There were no changes in food intake or metabolic rate, and only moderate differences in exercise performance. RNA-seq and microbiome analyses identified hypothalamic mRNAs and differentially populated gut bacteria, supporting future mechanistic analyses.

NUTRIENTS (2022)

Add to Collection

Correction Medicine, Research & Experimental

Voluntary wheel running complements microdystrophin gene therapy to improve muscle function in mdx mice (vol 21, pg 144, 2021)

Shelby E. Hamm, Daniel D. Fathalikhani, Katherine E. Bukovec, Adele K. Addington, Haiyan Zhang, Justin B. Perry, Ryan P. McMillan, Michael W. Lawlor, Mariah J. Prom, Mark A. Vanden Avond, Suresh N. Kumar, Kirsten E. Coleman, J. B. Dupont, David L. Mack, David A. Brown, Carl A. Morris, J. Patrick Gonzalez, Robert W. Grange

MOLECULAR THERAPY-METHODS & CLINICAL DEVELOPMENT (2021)

Add to Collection

Review Clinical Neurology

Diagnosis and management of Duchenne muscular dystrophy, part 1: diagnosis, and neuromuscular, rehabilitation, endocrine, and gastrointestinal and nutritional management

David J. Birnkrant, Katharine Bushby, Carla M. Bann, Susan D. Apkon, Angela Blackwell, David Brumbaugh, Laura E. Case, Paula R. Clemens, Stasia Hadjiyannakis, Shree Pandya, Natalie Street, Jean Tomezsko, Kathryn R. Wagner, Leanne M. Ward, David R. Weber

LANCET NEUROLOGY (2018)

Add to Collection

No Data Available

© Peeref 2019-2024. All rights reserved.