Cystamine-d
8 (hydrochloride) is intended for use as an internal standard for the quantification of cystamine by GC- or LC-MS. Cystamine is an organic disulfide that inhibits tissue transglutaminase (TGM2) with an IC
50 value of approximately 2.5 mM.
1 It is orally available and is neuroprotective in mouse models of Huntington’s disease.
2,3 Cystamine also inhibits caspase-3, increases intracellular glutathione, and reduces inflammation in a rat model of inflammatory bowel disease.
4
1. Smethurst, P.A., and Griffin, M. Measurement of tissue transglutaminase activity in a permeabilized cell system: Its regulation by Ca2+ and nucleotides Biochem. J. 313(pt 3),803-808(1996).
2. Dedeoglou, A., Kubilus, J.K., Jeitner, T.M., et al. Therapeutic effects of cystamine in a murine model of Huntington’s disease J. Neurosci. 22(20),8942-8950(2002).
3. Borrell-Pagès, M., Canals, K.P., Cordelièrs, F.P., et al. Cystamine and cysteamine increase brain levels of BDNF in Huntington disease via HSJ1b and transglutaminase J. Clin. Invest. 116(5),1410-1424(2006).
4. Elli, L., Ciulla, M.M., Busca, G., et al. Beneficial effects of treatment with transglutaminase inhibitor cystamine on the severity of inflammation in a rat model of inflammatory bowel disease Lab. Invest. 91(3),452-461(2011).
