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(2005) Cystathionine beta-synthase, a key enzyme for homocysteine metabolism, is preferentially expressed in the radial glia/astrocyte lineage of developing mouse CNS.
(2005) Cystathionine beta-synthase is enriched in the brains of Down's patients.
Reaction mechanism and regulation of cystathionine beta-synthase
. Biochim Biophys Acta 1647:30-35.
The formation of endogenous H[sub]2S needs cysteine as substrate, and mainly involves three enzymes: cystathionine beta-synthase
(CBS), cystathionine gamma-lyase (CSE), and 3-mercaptopyruvate sulfurtransferase (MPST).
Homocystinuria due to cystathionine beta-synthase
deficiency-the effects of betaine treatment in pyridoxine-responsive patients.
In homocystinuria caused by cystathionine beta-synthase
deficiency, circulating methionine is often increased, whereas homocystinuria resulting from a relative deficiency of the remethylation pathway is characterized by hypomethioninemia .