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Article

Calpain-1 ablation partially rescues disease-associated hallmarks in models of Machado-Joseph disease.

Human molecular genetics - 15 Apr 2020

Weber Jonasz J, Haas Eva, Maringer Yacine, Hauser Stefan, Casadei Nicolas L P, Chishti Athar H, Riess Olaf, Hübener-Schmid Jeannette

Abstract excerpt

Proteolytic fragmentation of polyglutamine-expanded ataxin-3 is a concomitant and modifier of the molecular pathogenesis of Machado-Joseph disease (MJD), the most common autosomal dominant cerebellar ataxia. Calpains, a group of calcium-dependent cysteine proteases, are important mediators of ataxin-3 cleavage and implicated in multiple neurodegenerative conditions. Pharmacologic and genetic approaches lowering...

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Source

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Identifiers

PMID: 31960910
DOI: 10.1093/hmg/ddaa010
PMCID: PMC7158375

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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Calpain-1 ablation partially rescues disease-associated hallmarks in models of Machado-Joseph disease.PMID 31960910Read ↗

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