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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Detalles Bibliográficos
Autores principales: Ichhaporia, Viraj P., Kim, Jieun, Kavdia, Kanisha, Vogel, Peter, Horner, Linda, Frase, Sharon, Hendershot, Linda M.
Formato: Online Artículo Texto
Lenguaje:English
Publicado: The Company of Biologists Ltd 2018
Materias:
Research Article
Acceso en línea:https://www.ncbi.nlm.nih.gov/pmc/articles/PMC5992605/
https://www.ncbi.nlm.nih.gov/pubmed/29666155
http://dx.doi.org/10.1242/dmm.033043

Internet

https://www.ncbi.nlm.nih.gov/pmc/articles/PMC5992605/
https://www.ncbi.nlm.nih.gov/pubmed/29666155
http://dx.doi.org/10.1242/dmm.033043

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