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Huntington's disease (HD)

Cystathionase (CTH)

Studies in mice and human samples suggest dietary supplementation with cysteine could help prevent neurotoxicity in patients with HD. The enzyme CTH generates cysteine from cystathionine. In mouse models of HD and in patient tissue samples, CTH levels were lower in HD-relevant brain regions than those in healthy mice or samples from healthy subjects. In a transgenic mouse model of HD, a cysteine-enriched diet delayed motor abnormalities, partially reverted loss of brain weight and extended survival, whereas a normal diet did not. Next steps could include investigating additional cysteine-related therapies in animal models.

SciBX 7(16); doi:10.1038/scibx.2014.467
Published online April 24, 2014

Unpatented; licensing status not applicable

Paul, B.D. et al. Nature; published online March 26, 2014;
Contact: Solomon H. Snyder, The Johns Hopkins University School of Medicine, Baltimore, Md.