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Cystamine-d8 (hydrochloride) is intended for use as an internal standard for the quantification of cystamine (Item No. 14882) by GC- or LC-MS. Cystamine is an organic disulfide that inhibits tissue transglutaminase (TGM2) with an IC50 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
WARNING This product is not for human or veterinary use.
1. 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. Therapeutic effects of cystamine in a murine model of Huntington’s disease. J. Neurosci. 22(20), 8942-8950 (2002).
3. Cystamine and cysteamine increase brain levels of BDNF in Huntington disease via HSJ1b and transglutaminase. J. Clin. Invest. 116(5), 1410-1424 (2006).
4. 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).