期刊
MUSCLE & NERVE
卷 35, 期 3, 页码 322-326出版社
JOHN WILEY & SONS INC
DOI: 10.1002/mus.20691
关键词
aggresome; endoplasmic reticulum stress; endoplasmic reticulum stress-associated degradation (EDRA); reducing-body myopathy; unfolded protein response
Reducing-body myopathy (RBM) is a rare myopathy characterized by the presence of unique sarcoplasmic inclusions called reducing bodies (RBs). We characterized the aggresomal features of RBs that contained gamma-tubulin, ubiquitin, and endoplasmic reticulum (ER) chaperones, together with a set of membrane proteins, in a family with hereditary RBM. Increased messenger ribonucleic acid and protein levels of a molecular chaperone, glucose-related protein 78, were also observed. These results suggest that the unfolded protein response caused by the accumulation of misfolded proteins in the endoplasmic reticulum plays an important role in the formation of RBs.
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