NEMF mutations that impair ribosome-associated quality control are associated with neuromuscular disease.

doi:10.1038/s41467-020-18327-6

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Article

NEMF mutations that impair ribosome-associated quality control are associated with neuromuscular disease.

Nature communications - 15 Sept 2020

Martin Paige B, Kigoshi-Tansho Yu, Sher Roger B, Ravenscroft Gianina, Stauffer Jennifer E, Kumar Rajesh, Yonashiro Ryo, Müller Tina, Griffith Christopher, Allen William, Pehlivan Davut, Harel Tamar, Zenker Martin, Howting Denise, Schanze Denny, Faqeih Eissa A, Almontashiri Naif A M, Maroofian Reza, Houlden Henry, Mazaheri Neda, Galehdari Hamid, Douglas Ganka, Posey Jennifer E, Ryan Monique, Lupski James R, Laing Nigel G, Joazeiro Claudio A P, Cox Gregory A

Abstract excerpt

A hallmark of neurodegeneration is defective protein quality control. The E3 ligase Listerin (LTN1/Ltn1) acts in a specialized protein quality control pathway-Ribosome-associated Quality Control (RQC)-by mediating proteolytic targeting of incomplete polypeptides produced by ribosome stalling, and Ltn1 mutation leads to neurodegeneration in mice. Whether neurodegeneration results from defective RQC and whether...

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PMID: 32934225
DOI: 10.1038/s41467-020-18327-6
PMCID: PMC7494853

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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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