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Article

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

Nature communications - 23 Jul 2018

Koyuncu Seda, Saez Isabel, Lee Hyun Ju, Gutierrez-Garcia Ricardo, Pokrzywa Wojciech, Fatima Azra, Hoppe Thorsten, Vilchez David

Abstract excerpt

Induced pluripotent stem cells (iPSCs) undergo unlimited self-renewal while maintaining their potential to differentiate into post-mitotic cells with an intact proteome. As such, iPSCs suppress the aggregation of polyQ-expanded huntingtin (HTT), the mutant protein underlying Huntington's disease (HD). Here we show that proteasome activity determines HTT levels, preventing polyQ-expanded aggregation in iPSCs from...

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Source

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Identifiers

PMID: 30038412
DOI: 10.1038/s41467-018-05320-3
PMCID: PMC6056416

Genes

UBR5
HTT

Provenance

PubMed metadata and Literature Corpus relationships. Corpus snapshot updated 7 Sept 2026. Manifest publication-graph-cohort-2026-09-07.1.

Source receipt: pubmed, retrieved 7 Sept 2026.

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