Article
Re-establishing ataxin-2 downregulates translation of mutant ataxin-3 and alleviates Machado-Joseph disease.
Brain : a journal of neurology - 1 Dec 2015
Nóbrega Clévio, Carmo-Silva Sara, Albuquerque David, Vasconcelos-Ferreira Ana, Vijayakumar Udaya-Geetha, Mendonça Liliana, Hirai Hirokazu, de Almeida Luís Pereira
Abstract excerpt
Machado-Joseph disease is a progressive neurodegenerative disorder associated with the polyQ-expanded ataxin-3 (encoded by ATXN3), for which no therapy is available. With the aim of clarifying the mechanism of neurodegeneration, we hypothesized that the abnormally long polyQ tract would interact aberrantly with ataxin-2 (encoded by ATXN2), another polyQ protein whose function has recently been linked to...
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