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Article

hMTH1 expression protects mitochondria from Huntington's disease-like impairment.

Neurobiology of disease - 1 Jan 2013

Ventura Ilenia, Russo Maria Teresa, De Nuccio Chiara, De Luca Gabriele, Degan Paolo, Bernardo Antonietta, Visentin Sergio, Minghetti Luisa, Bignami Margherita

Abstract excerpt

Huntington disease (HD) is a neurodegenerative disease caused by expansion of CAG repeats in the huntingtin (Htt) gene. The expression of hMTH1, the human hydrolase that degrades oxidized purine nucleoside triphosphates, grants protection in a chemical HD mouse model in which HD-like features are induced by the mitochondrial toxin 3-nitropropionic acid (3-NP). To further examine the relationship between oxidized...

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Identifiers

PMID: 22974734
DOI: 10.1016/j.nbd.2012.09.002
PMCID: PMC3507627

Genes

MFN1

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