Endoplasmic Reticulum-associated Degradation of Niemann-Pick C1: EVIDENCE FOR THE ROLE OF HEAT SHOCK PROTEINS AND IDENTIFICATION OF LYSINE RESIDUES THAT ACCEPT UBIQUITIN

Most cases with Niemann-Pick disease type C carry mutations in NPC1. Some of the mutations, including the most frequent I1061T, give rise to unstable proteins selected for endoplasmic reticulum-associated degradation. The purpose of the current study was to shed mechanistic insights into the degrada...

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Bibliographic Details
Main Authors: Nakasone, Naoe, Nakamura, Yuko S., Higaki, Katsumi, Oumi, Nao, Ohno, Kousaku, Ninomiya, Haruaki
Format: Artigo
Language:Inglês
Published: American Society for Biochemistry and Molecular Biology 2014
Subjects:
Molecular Bases of Disease
Online Access:https://ncbi.nlm.nih.gov/pmc/articles/PMC4094081/
https://ncbi.nlm.nih.gov/pubmed/24891511
https://ncbi.nlm.nih.govhttp://dx.doi.org/10.1074/jbc.M114.549915
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