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FKBP10 Depletion Enhances Glucocerebrosidase Proteostasis in Gaucher's Disease Fibroblasts

Lysosomal storage diseases (LSDs) are often caused by mutations compromising lysosomal enzyme folding in the endoplasmic reticulum (ER), leading to degradation and loss of function. Mass spectrometry analysis of Gaucher fibroblasts treated with mechanistically distinct molecules that increase LSD en...

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Bibliografiske detaljer
Main Authors: Ong, Derrick Sek Tong, Wang, Ya-Juan, Tan, Yun Lei, Yates, John R., Mu, Ting-Wei, Kelly, Jeffery W.
Format: Artigo
Sprog:Inglês
Udgivet: 2013
Fag:
Online adgang:https://ncbi.nlm.nih.gov/pmc/articles/PMC3624024/
https://ncbi.nlm.nih.gov/pubmed/23434032
https://ncbi.nlm.nih.govhttp://dx.doi.org/10.1016/j.chembiol.2012.11.014
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