SIL1, the endoplasmic-reticulum-localized BiP co-chaperone, plays a crucial role in maintaining skeletal muscle proteostasis and physiology

SIL1, the endoplasmic-reticulum-localized BiP co-chaperone, plays a crucial role in maintaining skeletal muscle proteostasis and physiology

Mutations in SIL1, a cofactor for the endoplasmic reticulum (ER)-localized Hsp70 chaperone, BiP, cause Marinesco-Sjögren syndrome (MSS), an autosomal recessive disorder. Using a mouse model, we characterized molecular aspects of the progressive myopathy associated with MSS. Proteomic profiling of qu...

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Bibliographic Details
Main Authors: Viraj P. Ichhaporia, Jieun Kim, Kanisha Kavdia, Peter Vogel, Linda Horner, Sharon Frase, Linda M. Hendershot
Format: Article
Language:English
Published: The Company of Biologists 2018-05-01
Series:Disease Models & Mechanisms
Subjects:
SIL1
Endoplasmic reticulum
Marinesco-Sjögren syndrome
Myopathy
Proteostasis collapse
PI3K-AKT-mTOR signaling
Online Access:http://dmm.biologists.org/content/11/5/dmm033043

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