FEATURES

FAMILY

cysteine synthase/cystathionine beta-synthase family

DESCRIPTION

Also known as CBS_HUMAN, CBS. Hydro-lyase catalyzing the first step of the transsulfuration pathway, where the hydroxyl group of L-serine is displaced by L-homocysteine in a beta-replacement reaction to form L-cystathionine, the precursor of L-cysteine. This catabolic route allows the elimination of L-methionine and the toxic metabolite L-homocysteine (PubMed:23981774, PubMed:20506325, PubMed:23974653). Also involved in the production of hydrogen sulfide, a gasotransmitter with signaling and cytoprotective effects on neurons (By similarity). Homotetramer.

EXTERNAL LINKS

uniprot
enzyme classification
PhosphoSitePlus

CBS

Mutations

Mutation(s) distribution

Mutations table