Coordinate regulation of mutant NPC1 degradation by selective ER autophagy and MARCH6-dependent ERAD

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.

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Autores principales:	Mark L. Schultz, Kelsey L. Krus, Susmita Kaushik, Derek Dang, Ravi Chopra, Ling Qi, Vikram G. Shakkottai, Ana Maria Cuervo, Andrew P. Lieberman
Formato:	article
Lenguaje:	EN
Publicado:	Nature Portfolio 2018
Materias:	Science Q
Acceso en línea:	https://doaj.org/article/fa59083b03654256906367e10286935a
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spelling	oai:doaj.org-article:fa59083b03654256906367e10286935a2021-12-02T17:33:18ZCoordinate regulation of mutant NPC1 degradation by selective ER autophagy and MARCH6-dependent ERAD10.1038/s41467-018-06115-22041-1723https://doaj.org/article/fa59083b03654256906367e10286935a2018-09-01T00:00:00Zhttps://doi.org/10.1038/s41467-018-06115-2https://doaj.org/toc/2041-1723Niemann-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.Mark L. SchultzKelsey L. KrusSusmita KaushikDerek DangRavi ChopraLing QiVikram G. ShakkottaiAna Maria CuervoAndrew P. LiebermanNature PortfolioarticleScienceQENNature Communications, Vol 9, Iss 1, Pp 1-13 (2018)
institution	DOAJ
collection	DOAJ
language	EN
topic	Science Q
spellingShingle	Science Q Mark L. Schultz Kelsey L. Krus Susmita Kaushik Derek Dang Ravi Chopra Ling Qi Vikram G. Shakkottai Ana Maria Cuervo Andrew P. Lieberman Coordinate regulation of mutant NPC1 degradation by selective ER autophagy and MARCH6-dependent ERAD
description	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.
format	article
author	Mark L. Schultz Kelsey L. Krus Susmita Kaushik Derek Dang Ravi Chopra Ling Qi Vikram G. Shakkottai Ana Maria Cuervo Andrew P. Lieberman
author_facet	Mark L. Schultz Kelsey L. Krus Susmita Kaushik Derek Dang Ravi Chopra Ling Qi Vikram G. Shakkottai Ana Maria Cuervo Andrew P. Lieberman
author_sort	Mark L. Schultz
title	Coordinate regulation of mutant NPC1 degradation by selective ER autophagy and MARCH6-dependent ERAD
title_short	Coordinate regulation of mutant NPC1 degradation by selective ER autophagy and MARCH6-dependent ERAD
title_full	Coordinate regulation of mutant NPC1 degradation by selective ER autophagy and MARCH6-dependent ERAD
title_fullStr	Coordinate regulation of mutant NPC1 degradation by selective ER autophagy and MARCH6-dependent ERAD
title_full_unstemmed	Coordinate regulation of mutant NPC1 degradation by selective ER autophagy and MARCH6-dependent ERAD
title_sort	coordinate regulation of mutant npc1 degradation by selective er autophagy and march6-dependent erad
publisher	Nature Portfolio
publishDate	2018
url	https://doaj.org/article/fa59083b03654256906367e10286935a
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Coordinate regulation of mutant NPC1 degradation by selective ER autophagy and MARCH6-dependent ERAD

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