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PMID: 11483494 Published · ppublish English Journal Article Research Support, Non-U.S. Gov't Research Support, U.S. Gov't, P.H.S.

Structure of human cystathionine beta-synthase: a unique pyridoxal 5'-phosphate-dependent heme protein.

The EMBO journal ·Vol. 20 ·No. 15 ·2001-08-01 ·Pages 3910-6

Meier M, Janosik M, Kery V, Kraus JP, Burkhard P

Abstract

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 an additional N-terminal heme binding site. This heme binding motif together with a spatially adjacent oxidoreductase active site motif could explain the regulation of its enzyme activity by redox changes.

MeSH Terms
Amino Acid Sequence Animals Binding Sites Cystathionine beta-Synthase/chemistry,genetics,metabolism Heme/metabolism Hemeproteins/chemistry,genetics,metabolism Humans Models, Molecular Molecular Sequence Data Mutagenesis Oxidoreductases/metabolism Protein Structure, Secondary Pyridoxal Phosphate/metabolism Rabbits
Chemicals
Hemeproteins Heme Pyridoxal Phosphate Oxidoreductases Cystathionine beta-Synthase
Authors & Affiliations
5 authors, click to expand affiliations / ORCID
Meier M
M.E.Müller Institute for Structural Biology, Biozentrum, University of Basel, Klingelbergstrasse 70, CH-4056 Basel, Switzerland.
Janosik M
Kery V
Kraus J P
Burkhard P
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Article Info
Journal
The EMBO journal
Abbr.
EMBO J
ISSN
0261-4189
DOI
10.1093/emboj/20.15.3910
Published
2001-08-01
Pages
3910-6
Language
English
Region
England
NLM ID
8208664
PMCID
PMC149156
Subset
IM
Databases
PDB
1JBQ
External Links
PubMed source DOI: 10.1093/emboj/20.15.3910 PMC Full Text View journal details
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