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

• Main Authors: Viraj P. Ichhaporia (Author), Jieun Kim (Author), Kanisha Kavdia (Author), Peter Vogel (Author), Linda Horner (Author), Sharon Frase (Author), Linda M. Hendershot (Author)
• Format: Book
• Published: The Company of Biologists, 2018-05-01T00:00:00Z.
• Subjects: article
• Online Access: Connect to this object online.

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 q...