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Alteration of the unfolded protein response modifies neurodegeneration in a mouse model of Marinesco–Sjögren syndrome

Endoplasmic reticulum (ER) stress has been linked to the onset and progression of many diseases. SIL1 is an adenine nucleotide exchange factor of the essential ER lumen chaperone HSPA5/BiP that senses ER stress and is involved in protein folding. Mutations in the Sil1 gene have been associated with...

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Detalles Bibliográficos
Autores principales: Zhao, Lihong, Rosales, Christine, Seburn, Kevin, Ron, David, Ackerman, Susan L.
Formato: Texto
Lenguaje:English
Publicado: Oxford University Press 2010
Materias:
Acceso en línea:https://www.ncbi.nlm.nih.gov/pmc/articles/PMC2792147/
https://www.ncbi.nlm.nih.gov/pubmed/19801575
http://dx.doi.org/10.1093/hmg/ddp464