Cystathionine γ-lyase deficiency mediates neurodegeneration in Huntington’s disease

Bindu D. Paul, Juan I. Sbodio, Risheng Xu, M. Scott Vandiver, Jiyoung Y. Cha, Adele M. Snowman and Solomon H. Snyder ()
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Bindu D. Paul: Johns Hopkins University School of Medicine
Juan I. Sbodio: Johns Hopkins University School of Medicine
Risheng Xu: Johns Hopkins University School of Medicine
M. Scott Vandiver: Johns Hopkins University School of Medicine
Jiyoung Y. Cha: Johns Hopkins University School of Medicine
Adele M. Snowman: Johns Hopkins University School of Medicine
Solomon H. Snyder: Johns Hopkins University School of Medicine

Nature, 2014, vol. 509, issue 7498, 96-100

Abstract: Cystathionine γ-lyase, which is responsible for the production of cysteine, is decreased in the striatum and cortex of mouse models of Huntington’s disease and in patients with Huntington’s disease, and cysteine supplementation in diet and drinking water partly rescues the phenotype and the diminished longevity of the mouse model.

Date: 2014
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DOI: 10.1038/nature13136

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