CHIP mutations affect the heat shock response differently in human fibroblasts and iPSC-derived neurons

• Main Authors: S. Schuster (Author), E. Heuten (Author), A. Velic (Author), J. Admard (Author), M. Synofzik (Author), S. Ossowski (Author), B. Macek (Author), S. Hauser (Author), L. Schöls (Author)
• Format: Book
• Published: The Company of Biologists, 2020-10-01T00:00:00Z.
• Subjects: article
• Online Access: Connect to this object online.

C-terminus of HSC70-interacting protein (CHIP) encoded by the gene STUB1 is a co-chaperone and E3 ligase that acts as a key regulator of cellular protein homeostasis. Mutations in STUB1 cause autosomal recessive spinocerebellar ataxia type 16 (SCAR16) with widespread neurodegeneration manifesting as...