e-Article
Coordinate regulation of mutant NPC1 degradation by selective ER autophagy and MARCH6-dependent ERAD
Document Type
article
Author
Source
Nature Communications, Vol 9, Iss 1, Pp 1-13 (2018)
Subject
Language
English
ISSN
2041-1723
Abstract
Niemann-Pick type C1 disease is most commonly caused by the allele NPC1 I1061T, which is misfolded in the ER and rapidly degraded by the ubiquitin proteasome system. Here the authors show that the I1061T mutant is also degraded by ER-phagy.