Genetic compensation for sarcoglycan loss by integrin α7β1 in muscle
نویسندگان
چکیده
منابع مشابه
Loss of sarcolemma nNOS in sarcoglycan-deficient muscle.
nNOS, anchored to the sarcolemma through its interactions with the dystrophin-glycoprotein complex, is dramatically reduced in dystrophin-deficient mdx mice and Duchenne muscular dystrophy patients. Recent evidence suggests that loss of nNOS in dystrophin-deficient muscle may contribute significantly to the progression of muscle pathology through a variety of mechanisms. To investigate whether ...
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The lack of a mutant phenotype in homozygous mutant individuals' due to compensatory gene expression triggered upstream of protein function has been identified as genetic compensation. Whilst this intriguing process has been recognized in zebrafish, the presence of homozygous loss of function mutations in healthy human individuals suggests that compensation may not be restricted to this model. ...
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The α7β1 integrin was originally identified and isolated from differentiating skeletal muscle and shown to be a laminin-binding protein (Song et al. (1992) J. Cell Biol. 117, 643-657). Expression of the α7 gene and protein are developmentally regulated during skeletal muscle differentiation and have been used to identify cells at distinct stages of the myogenic lineage (George-Weinstein et al. ...
متن کاملThe sarcoglycan complex in skeletal muscle.
In skeletal muscle, the dystrophin-associated glycoprotein complex forms a link between the actin cytoskeleton and the extracellular matrix that is critical for muscle integrity. Within this complex resides the sarcoglycan subcomplex, which consists of four transmembrane glycoproteins (alpha-, beta-, gamma-, and delta-sarcoglycan). During assembly, beta-sarcoglycan tightly associates with delta...
متن کاملA Functional Role for Specific Spliced Variants of the α7β1 Integrin in Acetylcholine Receptor Clustering
The clustering of acetylcholine receptors (AChR) on skeletal muscle fibers is an early event in the formation of neuromuscular junctions. Recent studies show that laminin as well as agrin can induce AChR clustering. Since the alpha7beta1 integrin is a major laminin receptor in skeletal muscle, we determined if this integrin participates in laminin and/or agrin-induced AChR clustering. The alter...
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ژورنال
عنوان ژورنال: Journal of Cell Science
سال: 2004
ISSN: 1477-9137,0021-9533
DOI: 10.1242/jcs.01234