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Identification of the calpain-generated toxic fragment of ataxin-3 protein provides new avenues for therapy of Machado-Joseph disease| Spinocerebellar ataxia type 3.

Neuropathology and applied neurobiology - 1 Feb 2022

Simões Ana Teresa, Carmona Vítor, Duarte-Neves Joana, Cunha-Santos Janete, Pereira de Almeida Luís

Abstract excerpt

AIMS: Machado-Joseph disease (MJD) is the most frequent dominantly inherited cerebellar ataxia worldwide. Expansion of a CAG trinucleotide in the MJD1 gene translates into a polyglutamine tract within ataxin-3, which upon proteolysis may lead to MJD. The aim of this work was to understand the in vivo contribution of calpain proteases to the pathogenesis of MJD. Therefore, we investigated (a) the calpain cleavage...

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PMID: 34273111
DOI: 10.1111/nan.12748

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Identification of the calpain-generated toxic fragment of ataxin-3 protein provides new avenues for therapy of Machado-Joseph disease| Spinocerebellar ataxia type 3.PMID 34273111Read ↗

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