The ubiquitin ligase UBR5 suppresses proteostasis collapse in pluripotent stem cells from Huntington’s disease patients

The ubiquitin ligase UBR5 suppresses proteostasis collapse in pluripotent stem cells from Huntington’s disease patients

Induced pluripotent stem cells (iPSCs) suppress the aggregation of Huntington’s disease (HD) polyQ-expanded huntingtin (HTT). Here the authors show that proteasome activity determines the levels of mutant HTT in HD-iPSCs and find that UBR5 is a modulator of super-vigilant proteostasis of iPSCs.

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Autores principales: Seda Koyuncu, Isabel Saez, Hyun Ju Lee, Ricardo Gutierrez-Garcia, Wojciech Pokrzywa, Azra Fatima, Thorsten Hoppe, David Vilchez
Formato: article
Lenguaje:EN
Publicado: Nature Portfolio 2018
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Acceso en línea:https://doaj.org/article/3208ae5c34064dfaa117ff00fd735b20
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spelling oai:doaj.org-article:3208ae5c34064dfaa117ff00fd735b202021-12-02T15:33:37ZThe ubiquitin ligase UBR5 suppresses proteostasis collapse in pluripotent stem cells from Huntington’s disease patients10.1038/s41467-018-05320-32041-1723https://doaj.org/article/3208ae5c34064dfaa117ff00fd735b202018-07-01T00:00:00Zhttps://doi.org/10.1038/s41467-018-05320-3https://doaj.org/toc/2041-1723Induced pluripotent stem cells (iPSCs) suppress the aggregation of Huntington’s disease (HD) polyQ-expanded huntingtin (HTT). Here the authors show that proteasome activity determines the levels of mutant HTT in HD-iPSCs and find that UBR5 is a modulator of super-vigilant proteostasis of iPSCs.Seda KoyuncuIsabel SaezHyun Ju LeeRicardo Gutierrez-GarciaWojciech PokrzywaAzra FatimaThorsten HoppeDavid VilchezNature PortfolioarticleScienceQENNature Communications, Vol 9, Iss 1, Pp 1-22 (2018)
institution DOAJ
collection DOAJ
language EN
topic Science
Q
spellingShingle Science
Q
Seda Koyuncu
Isabel Saez
Hyun Ju Lee
Ricardo Gutierrez-Garcia
Wojciech Pokrzywa
Azra Fatima
Thorsten Hoppe
David Vilchez
The ubiquitin ligase UBR5 suppresses proteostasis collapse in pluripotent stem cells from Huntington’s disease patients
description Induced pluripotent stem cells (iPSCs) suppress the aggregation of Huntington’s disease (HD) polyQ-expanded huntingtin (HTT). Here the authors show that proteasome activity determines the levels of mutant HTT in HD-iPSCs and find that UBR5 is a modulator of super-vigilant proteostasis of iPSCs.
format article
author Seda Koyuncu
Isabel Saez
Hyun Ju Lee
Ricardo Gutierrez-Garcia
Wojciech Pokrzywa
Azra Fatima
Thorsten Hoppe
David Vilchez
author_facet Seda Koyuncu
Isabel Saez
Hyun Ju Lee
Ricardo Gutierrez-Garcia
Wojciech Pokrzywa
Azra Fatima
Thorsten Hoppe
David Vilchez
author_sort Seda Koyuncu
title The ubiquitin ligase UBR5 suppresses proteostasis collapse in pluripotent stem cells from Huntington’s disease patients
title_short The ubiquitin ligase UBR5 suppresses proteostasis collapse in pluripotent stem cells from Huntington’s disease patients
title_full The ubiquitin ligase UBR5 suppresses proteostasis collapse in pluripotent stem cells from Huntington’s disease patients
title_fullStr The ubiquitin ligase UBR5 suppresses proteostasis collapse in pluripotent stem cells from Huntington’s disease patients
title_full_unstemmed The ubiquitin ligase UBR5 suppresses proteostasis collapse in pluripotent stem cells from Huntington’s disease patients
title_sort ubiquitin ligase ubr5 suppresses proteostasis collapse in pluripotent stem cells from huntington’s disease patients
publisher Nature Portfolio
publishDate 2018
url https://doaj.org/article/3208ae5c34064dfaa117ff00fd735b20
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