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

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...

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Detalles Bibliográficos
Autores principales: Schuster, S., Heuten, E., Velic, A., Admard, J., Synofzik, M., Ossowski, S., Macek, B., Hauser, S., Schöls, L.
Formato: Online Artículo Texto
Lenguaje:English
Publicado: The Company of Biologists Ltd 2020
Materias:
Acceso en línea:https://www.ncbi.nlm.nih.gov/pmc/articles/PMC7578354/
https://www.ncbi.nlm.nih.gov/pubmed/33097556
http://dx.doi.org/10.1242/dmm.045096