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Cystamine and YAC128 mice

Submitted Sat, 07/09/2005
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Cystamine's effects in mitigating HD appear to be indirect.
This is the third in a series of three updates about cystamine in the HD mouse models. The researchers studied the YAC128 mice instead of the R6/2 mice. Although the evidence shows that cystamine acts as a treatment in various mouse models of HD, the mechanism in unknown, and the effect appears to be indirect.
Pinto, Van Raamsdonk, Leavitt, Hayden, Jeitner, Thaler, Krasnikov, and Cooper
Treatment of YAC128 mice and their wild-type littermates with cystamine does not lead to its accumulation in plasma or brain: implications for the treatment of Huntington disease.
Cystamine is beneficial to Huntington disease (HD) transgenic mice. To elucidate the mechanism, cystamine metabolites were determined in brain and plasma of cystamine-treated mice. A major route for cystamine metabolism is thought to be: cystamine --> cysteamine --> hypotaurine --> taurine. Here we describe an HPLC system with coulometric detection that can rapidly measure underivatized cystamine, cysteamine and hypotaurine, as well as cysteine and glutathione in the same deproteinized tissue sample. A method is also described for the coulometric estimation of taurine as its isoindole-sulfonate derivative. Using this new methodology we showed that cystamine and cysteamine are undetectable (
J Neurochem. 2005 Jun 30