Mutant glucocerebrosidase in Gaucher disease recruits Hsp27 to the Hsp90 chaperone complex for proteasomal degradation

Gaucher disease is caused by mutations of the GBA1 gene, which encodes the lysosomal anchored gluococerebrosidase (GCase). GBA1 mutations commonly result in protein misfolding, abnormal chaperone recognition, and premature degradation, but are less likely to affect catalytic activity. In the present...

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Publicado en:Proc Natl Acad Sci U S A
Autores principales: Yang, Chunzhang, Wang, Herui, Zhu, Dongwang, Hong, Christopher S., Dmitriev, Pauline, Zhang, Chao, Li, Yan, Ikejiri, Barbara, Brady, Roscoe O., Zhuang, Zhengping
Formato: Artigo
Lenguaje:Inglês
Publicado: National Academy of Sciences 2015
Materias:
Biological Sciences
Acceso en línea:https://ncbi.nlm.nih.gov/pmc/articles/PMC4313839/
https://ncbi.nlm.nih.gov/pubmed/25583479
https://ncbi.nlm.nih.govhttp://dx.doi.org/10.1073/pnas.1424288112
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