(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 [2].