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Article

Toxic effects of mutant huntingtin in axons are mediated by its proline-rich domain.

Brain : a journal of neurology - 3 Jun 2024

Brady Scott T, Mesnard-Hoaglin Nichole A, Mays Sarah, Priego Mercedes, Dziechciowska Joanna, Morris Sarah, Kang Minsu, Tsai Ming Ying, Purks Jennifer L, Klein Alison, Gaona Angelica, Melloni Alexandra, Connors Theresa, Hyman Bradley, Song Yuyu, Morfini Gerardo A

Abstract excerpt

Huntington's disease results from expansion of a polyglutamine tract (polyQ) in mutant huntingtin (mHTT) protein, but mechanisms underlying polyQ expansion-mediated toxic gain-of-mHTT function remain elusive. Here, deletion and antibody-based experiments revealed that a proline-rich domain (PRD) adjacent to the polyQ tract is necessary for mHTT to inhibit fast axonal transport and promote axonal pathology in...

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Identifiers

PMID: 37633260
DOI: 10.1093/brain/awad280
PMCID: PMC11146425

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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