Unfolded protein response and aggresome formation in hereditary reducing-body myopathy
✍ Scribed by Teerin Liewluck; Yukiko K. Hayashi; Maki Ohsawa; Rumi Kurokawa; Masako Fujita; Satoru Noguchi; Ikuya Nonaka; Ichizo Nishino
- Book ID
- 102533756
- Publisher
- John Wiley and Sons
- Year
- 2007
- Tongue
- English
- Weight
- 210 KB
- Volume
- 35
- Category
- Article
- ISSN
- 0148-639X
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✦ Synopsis
Abstract
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 γ–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. Muscle Nerve, 2006