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Unfolded protein response and aggresome formation in hereditary reducing‐body myopathy
Author(s) -
Liewluck Teerin,
Hayashi Yukiko K.,
Ohsawa Maki,
Kurokawa Rumi,
Fujita Masako,
Noguchi Satoru,
aka Ikuya,
Nishino Ichizo
Publication year - 2007
Publication title -
muscle and nerve
Language(s) - English
Resource type - Journals
SCImago Journal Rank - 1.025
H-Index - 145
eISSN - 1097-4598
pISSN - 0148-639X
DOI - 10.1002/mus.20691
Subject(s) - endoplasmic reticulum , aggresome , unfolded protein response , chaperone (clinical) , myopathy , protein folding , microbiology and biotechnology , chemical chaperone , sarcoplasm , chemistry , endoplasmic reticulum associated protein degradation , biology , ubiquitin , biochemistry , medicine , genetics , gene , pathology
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