Function and Biology

Human cystathionine beta-synthase (CBS) p.P49L delta409-551 variant

Source organism: Homo sapiens
Biochemical function: cystathionine beta-synthase activity
Biological process: cysteine biosynthetic process via cystathionine
Cellular component: cytoplasm

EC 4.2.1.22: Cystathionine beta-synthase

Reaction catalysed:
L-serine + L-homocysteine = L-cystathionine + H(2)O
Systematic name:
L-serine hydro-lyase (adding homocysteine; L-cystathionine-forming)
Alternative Name(s):
  • Beta-thionase
  • Cysteine synthase
  • L-serine hydro-lyase (adding homocysteine)
  • Methylcysteine synthase
  • Serine sulfhydrase
  • Serine sulfhydrylase

Sequence family

Pfam Protein family (Pfam)
PF00291
Domain description: Pyridoxal-phosphate dependent enzyme
Occurring in:
  1. Cystathionine beta-synthase
The deposited structure of PDB entry 5mms contains 6 copies of Pfam domain PF00291 (Pyridoxal-phosphate dependent enzyme) in Cystathionine beta-synthase. Showing 1 copy in chain D.

InterPro InterPro annotations
IPR036052
Domain description: Tryptophan synthase beta chain-like, PALP domain superfamily
Occurring in:
  1. Cystathionine beta-synthase
IPR001216
Domain description: Cysteine synthase/cystathionine beta-synthase, pyridoxal-phosphate attachment site
Occurring in:
  1. Cystathionine beta-synthase
IPR001926
Domain description: Tryptophan synthase beta chain-like, PALP domain
Occurring in:
  1. Cystathionine beta-synthase
IPR005857
Domain description: Cystathionine beta-synthase
Occurring in:
  1. Cystathionine beta-synthase

Structure domain

CATH CATH domain
3.40.50.1100
Class: Alpha Beta
Architecture: 3-Layer(aba) Sandwich
Topology: Rossmann fold
Homology: Rossmann fold
Occurring in:
  1. Cystathionine beta-synthase
The deposited structure of PDB entry 5mms contains 12 copies of CATH domain 3.40.50.1100 (Rossmann fold) in Cystathionine beta-synthase. Showing 2 copies in chain D.