Muscle histology in becker muscular dystrophy

Kaido, Misako; Arahata, Kiichi; Hoffman, Eric P.; Nonaka, Ikuya; Sugita, Hideo

doi:10.1002/mus.880141105

Cited by 27 publications

(16 citation statements)

References 15 publications

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“…This has been found in both humans (Andersen, 2003) and murine models (Kanda and Hashizume, 1989; Rowan et al, 2011). Certainly, fiber type grouping is not very extensive in most muscular dystrophies, but the occurrence of smaller fiber groups was reported for samples from Becker muscular dystrophy (ten Houten and De Visser, 1984; Kaido et al, 1991) and also Duchenne muscular dystrophy (Engel and Ozawa, 2004). Another fiber type-related alteration is the co-expression of multiple myosin heavy chain isoforms, which is again indicative of sarcopenia (Andersen et al, 1999; Patterson et al, 2006; Rowan et al, 2012) and was also observed in Duchenne muscular dystrophy (Marini et al, 1991).…”

Section: Common Features In Aging and Dystrophy With Respect To The Nmjmentioning

confidence: 98%

Degeneration of Neuromuscular Junction in Age and Dystrophy

Rudolf

Khan

Labeit

et al. 2014

Front. Aging Neurosci.

159

141

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Functional denervation is a hallmark of aging sarcopenia as well as of muscular dystrophy. It is thought to be a major factor reducing skeletal muscle mass, particularly in the case of sarcopenia. Neuromuscular junctions (NMJs) serve as the interface between the nervous and skeletal muscular systems, and thus they may receive pathophysiological input of both pre- and post-synaptic origin. Consequently, NMJs are good indicators of motor health on a systemic level. Indeed, upon sarcopenia and dystrophy, NMJs morphologically deteriorate and exhibit altered characteristics of primary signaling molecules, such as nicotinic acetylcholine receptor and agrin. Since a remarkable reversibility of these changes can be observed by exercise, there is significant interest in understanding the molecular mechanisms underlying synaptic deterioration upon aging and dystrophy and how synapses are reset by the aforementioned treatments. Here, we review the literature that describes the phenomena observed at the NMJ in sarcopenic and dystrophic muscle as well as to how these alterations can be reversed and to what extent. In a second part, the current information about molecular machineries underlying these processes is reported.

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Section: Common Features In Aging and Dystrophy With Respect To The Nmjmentioning

confidence: 98%

Degeneration of Neuromuscular Junction in Age and Dystrophy

Rudolf

Khan

Labeit

et al. 2014

Front. Aging Neurosci.

159

141

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“…Muscular dystrophies are classic examples of muscle‐fiber loss by necrosis 7, 24, 27, 85, 114. It is not conclusively known whether apoptosis and necrosis in muscular dystrophies represent two independently initiated events or whether necrosis succeeds apoptosis 198.…”

Section: Apoptosis In Neuromuscular Disordersmentioning

confidence: 99%

Muscle‐fiber apoptosis in neuromuscular diseases

Tews

2005

Muscle and Nerve

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Muscle-fiber loss is a characteristic of many progressive neuromuscular disorders. Over the past decade, identification of a growing number of apoptosis-associated factors and events in pathological skeletal muscle provided increasing evidence that apoptotic cell-death mechanisms account significantly for muscle-fiber atrophy and loss in a wide spectrum of neuromuscular disorders. It became obvious that there is not one specific pathway for muscle fibers to undergo apoptotic degradation. In contrast, certain neuromuscular diseases seem to involve characteristic expression patterns of apoptosis-related factors and pathways. Furthermore, there are some characteristics of muscle-fiber apoptosis that rely on the muscle fiber itself as an extremely specified cell type. Multinucleated muscle fibers with successive muscle-fiber segments controlled by individual nuclei display some specifics different from apoptosis of mononucleated cells. This review focuses on the expression patterns of apoptosis-associated factors in different primary and secondary neuromuscular disorders and gives a synopsis of current knowledge.

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“…In BMD, skeletal muscle pathology is similar but milder . In younger patients (15 years old), the muscle shows signs of muscle necrosis and regeneration, whereas in older patients (>15 years old), centralized nuclei, split fibres, hypertrophic muscle fibres, and fibrosis are observed …”

Section: Introductionmentioning

confidence: 97%

Comparative proteomic analyses of Duchenne muscular dystrophy and Becker muscular dystrophy muscles: changes contributing to preserve muscle function in Becker muscular dystrophy patients

Capitanio

Moriggi

Torretta

et al. 2020

J cachexia sarcopenia muscle

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Background Duchenne muscular dystrophy (DMD) and Becker muscular dystrophy (BMD) are characterized by muscle wasting leading to loss of ambulation in the first or third decade, respectively. In DMD, the lack of dystrophin hampers connections between intracellular cytoskeleton and cell membrane leading to repeated cycles of necrosis and regeneration associated with inflammation and loss of muscle ordered structure. BMD has a similar muscle phenotype but milder. Here, we address the question whether proteins at variance in BMD compared with DMD contribute to the milder phenotype in BMD, thus identifying a specific signature to be targeted for DMD treatment. Methods Proteins extracted from skeletal muscle from DMD/BMD patients and young healthy subjects were either reduced and solubilized prior two-dimensional difference in gel electrophoresis/mass spectrometry differential analysis or tryptic digested prior label-free liquid chromatography with tandem mass spectrometry. Statistical analyses of proteins and peptides were performed by DeCyder and Perseus software and protein validation and verification by immunoblotting. Results Proteomic results indicate minor changes in the extracellular matrix (ECM) protein composition in BMD muscles with retention of mechanotransduction signalling, reduced changes in cytoskeletal and contractile proteins. Conversely, in DMD patients, increased levels of several ECM cytoskeletal and contractile proteins were observed whereas some proteins of fast fibres and of Z-disc decreased. Detyrosinated alpha-tubulin was unchanged in BMD and increased in DMD although neuronal nitric oxide synthase was unchanged in BMD and greatly reduced in DMD. Metabolically, the tissue is characterized by a decrement of anaerobic metabolism both in DMD and BMD compared with controls, with increased levels of the glycogen metabolic pathway in BMD. Oxidative metabolism is severely compromised in DMD with impairment of malate shuttle; conversely, it is active in BMD supporting the tricarboxylic acid cycle and respiratory chain. Adipogenesis characterizes DMD, whereas proteins involved in fatty acids beta-oxidation are increased in BMD. Proteins involved in protein/amino acid metabolism, cell development, calcium handling, endoplasmic reticulum/sarcoplasmic reticulum stress response, and inflammation/immune response were increased in DMD. Both disorders are characterized by the impairment of N-linked protein glycosylation in the endoplasmic reticulum. Authophagy was decreased in DMD whereas it was retained in BMD. Conclusions The mechanosensing and metabolic disruption are central nodes of DMD/BMD phenotypes. The ECM proteome composition and the metabolic rewiring in BMD lead to preservation of energy levels supporting autophagy and cell renewal, thus promoting the retention of muscle function. Conversely, DMD patients are characterized by extracellular and cytoskeletal O R I G I N A L A R T I C L E This is an open access article under the terms of the Creative Commons Attribution License, which permits use, di...

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Muscle histology in becker muscular dystrophy

Cited by 27 publications

References 15 publications

Degeneration of Neuromuscular Junction in Age and Dystrophy

Degeneration of Neuromuscular Junction in Age and Dystrophy

Muscle‐fiber apoptosis in neuromuscular diseases

Comparative proteomic analyses of Duchenne muscular dystrophy and Becker muscular dystrophy muscles: changes contributing to preserve muscle function in Becker muscular dystrophy patients

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