Transglutaminase inhibitor. Attenuates cell death induced by 3-nitropropionic acid
(Cat. No. 4849) in Huntington's disease (HD) striatal cells. Also decreases aggregated and cross-linked huntingtin in transfected cells, and increases brain levels of BDNF. Shown to extend survival and improve motor performance in transgenic HD mice. Neuroprotective.
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Cystamine and cysteamine increase brain levels of BDNF in Huntington disease via HSJ1b and transglutaminase.
Borrell-Pages et al.
Therapeutic effects of cystamine in a murine model of Huntington's disease.
Dedeoglu et al.
Cystamine and cysteamine prevent 3-NP-induced mitochondrial depolarization of Huntington's disease knock-in striatal cells.
Mao et al.