Ontology highlight
ABSTRACT:
SUBMITTER: Meier M
PROVIDER: S-EPMC149156 | biostudies-literature | 2001 Aug
REPOSITORIES: biostudies-literature
Meier M M Janosik M M Kery V V Kraus J P JP Burkhard P P
The EMBO journal 20010801 15
Cystathionine beta-synthase (CBS) is a unique heme- containing enzyme that catalyzes a pyridoxal 5'-phosphate (PLP)-dependent condensation of serine and homocysteine to give cystathionine. Deficiency of CBS leads to homocystinuria, an inherited disease of sulfur metabolism characterized by increased levels of the toxic metabolite homocysteine. Here we present the X-ray crystal structure of a truncated form of the enzyme. CBS shares the same fold with O-acetylserine sulfhydrylase but it contains ...[more]