Fast muscle fibers are preferentially affected in Duchenne muscular dystrophy

Webster, Cecelia; Silberstein, L.; Hays, Arthur P.; Blau, Helen M.

doi:10.1016/0092-8674(88)90463-1

Cited by 530 publications

(431 citation statements)

References 40 publications

Supporting

Mentioning

395

Contrasting

Unclassified

Order By: Relevance

“…Thus, reductions in nNOSμ may promote more fatiguesusceptible muscles in dystrophy. Also, type IIx fibers are more susceptible to degeneration and are preferentially lost in DMD patients (Webster et al 1988;Smerdu et al 1994). Thus, another consequence of nNOSμ reduction may be to accelerate muscle wasting, thus indirectly decreasing exercise tolerance.…”

Section: Nnosμ Function In Neuromuscular Diseasementioning

confidence: 99%

nNOS regulation of skeletal muscle fatigue and exercise performance

Percival

2011

Biophys Rev

View full text Add to dashboard Cite

Neuronal nitric oxide synthases (nNOS) are Ca 2+ /calmodulin-activated enzymes that synthesize the gaseous messenger nitric oxide (NO). nNOSμ and the recently described nNOSβ, both spliced nNOS isoforms, are important enzymatic sources of NO in skeletal muscle, a tissue long considered to be a paradigmatic system for studying NO-dependent redox signaling. nNOS is indispensable for skeletal muscle integrity and contractile performance, and deregulation of nNOSμ signaling is a common pathogenic feature of many neuromuscular diseases. Recent evidence suggests that both nNOSμ and nNOSβ regulate skeletal muscle size, strength, and fatigue resistance, making them important players in exercise performance. nNOSμ acts as an activity sensor and appears to assist skeletal muscle adaptation to new functional demands, particularly those of endurance exercise. Prolonged inactivity leads to nNOS-mediated muscle atrophy through a FoxO-dependent pathway. nNOS also plays a role in modulating exercise performance in neuromuscular disease. In the mdx mouse model of Duchenne muscular dystrophy, defective nNOS signaling is thought to restrict contractile capacity of working muscle in two ways: loss of sarcolemmal nNOSμ causes excessive ischemic damage while residual cytosolic nNOSμ contributes to hypernitrosylation of the ryanodine receptor, causing pathogenic Ca 2+ leak. This defect in Ca 2+ handling promotes muscle damage, weakness, and fatigue. This review addresses these recent advances in the understanding of nNOS-dependent redox regulation of skeletal muscle function and exercise performance under physiological and neuromuscular disease conditions.

show abstract

Section: Nnosμ Function In Neuromuscular Diseasementioning

confidence: 99%

nNOS regulation of skeletal muscle fatigue and exercise performance

Percival

2011

Biophys Rev

View full text Add to dashboard Cite

show abstract

“…Subsequently, bipolar cells that stain intracellularly for sarcomeric myosin appear. The bipolar cells then fuse to form multinucleated structures that stain intracellularly for sarcomeric myosin, fast-skeletal muscle myosin (MY-32, Sigma; Naumann and Pette, 1994), myosin heavy chain (ALD-58, DSHB; Shafiq et al, 1984), and human fast myosin (A4.74, DSHB; Webster et al, 1988). Skeletal structures undergoing myogenesis are further identified by their elongated structure, multiple nuclei, cross-striations, and spontaneous contractility (Pate et al, 1993;Rogers et al, 1995;Young, 2000;Young et al, 1992aYoung et al, , 1993Young et al, , 1995Young et al, , 1998aYoung et al, ,1998b.…”

Section: Insulin/dexamethasone Bioassaymentioning

confidence: 99%

Human reserve pluripotent mesenchymal stem cells are present in the connective tissues of skeletal muscle and dermis derived from fetal, adult, and geriatric donors

et al. 2001

View full text Add to dashboard Cite

This study details the profile of 13 cell surface cluster differentiation markers on human reserve stem cells derived from connective tissues. Stem cells were isolated from the connective tissues of dermis and skeletal muscle derived from fetal, mature, and geriatric humans. An insulin/dexamethasone phenotypic bioassay was used to determine the identity of the stem cells from each population. All populations contained lineage-committed myogenic, adipogenic, chondrogenic, and osteogenic progenitor stem cells as well as lineageuncommitted pluripotent stem cells capable of forming muscle, adipocytes, cartilage, bone, fibroblasts, and endothelial cells. Flow cytometric analysis of adult stem cell populations revealed positive staining for CD34 and CD90 and negative staining for CD3, CD4, CD8, CD11c, CD33, CD36, CD38, CD45, CD117, Glycophorin-A, and HLA DR-II. Anat Rec 264: [51][52][53][54][55][56][57][58][59][60][61][62] 2001.

show abstract

“…Frozen muscle sections were subjected to immunoperoxidase histochemistry by using antibodies A4.840 (MHCslow) (Webster et al, 1988) or N3.36 (MHCperi) (Cho et al, 1993) …”

Section: Fiber Typingmentioning

confidence: 99%

TnIfast IRE enhancer: Multistep developmental regulation during skeletal muscle fiber type differentiation

Hallauer

Hastings

2002

Developmental Dynamics

View full text Add to dashboard Cite

To identify developmental steps leading to adult skeletal muscle fiber-type-specific gene expression, we carried out transgenic mouse studies of the IRE enhancer of the quail TnIfast gene. Histochemical analysis of IRE/herpesvirus tk promoter/␤-galactosidase reporter transgene expression in adult muscle directly demonstrated IRE-driven fast vs. slow fiber-type specificity, and IIB>IIX>IIA differential expression among the fast fiber types: patterns similar to those of native-promoter TnIfast constructs. These tissue-and cell-type specificities are autonomous to the IRE and do not depend on interactions with a muscle gene promoter. Developmental studies showed that the adult pattern of IRE-driven transgene expression emerges in three steps: (1) activation during the formation of primary embryonic (presumptive slow) muscle fibers; (2) activation, to markedly higher levels, during formation of secondary (presumptive fast) fibers, and (3) differential augmentation of expression during early postnatal maturation of the IIB, IIX, IIA fast fiber types. These results provide insight into the roles of gene activation and gene repression mechanisms in fiber-type specificity and can account for apparently disparate results obtained in previous studies of TnI isoform expression in development. Each of the three IRE-driven developmental steps is spatiotemporally associated with a different major regulatory event at the fast myosin heavy chain gene cluster, suggesting that diverse muscle gene families respond to common, or tightly integrated, regulatory signals during multiple steps of muscle fiber differentiation.

show abstract

Fast muscle fibers are preferentially affected in Duchenne muscular dystrophy

Cited by 530 publications

References 40 publications

nNOS regulation of skeletal muscle fatigue and exercise performance

nNOS regulation of skeletal muscle fatigue and exercise performance

Human reserve pluripotent mesenchymal stem cells are present in the connective tissues of skeletal muscle and dermis derived from fetal, adult, and geriatric donors

TnIfast IRE enhancer: Multistep developmental regulation during skeletal muscle fiber type differentiation

Contact Info

Product

Resources

About