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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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Publicat a:Proc Natl Acad Sci U S A
Autors principals: Yang, Chunzhang, Wang, Herui, Zhu, Dongwang, Hong, Christopher S., Dmitriev, Pauline, Zhang, Chao, Li, Yan, Ikejiri, Barbara, Brady, Roscoe O., Zhuang, Zhengping
Format: Artigo
Idioma:Inglês
Publicat: National Academy of Sciences 2015
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Accés en línia: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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