Molybdenum cofactor (Moco) biosynthesis (WP4507)

This pathway visualises the Moco biosynthesis, which is used as a cofactor for several enzymes. Several diseases (MoCD type A, B and C) are clinically very similar to sulphite oxidase (SO) deficiency (see [] for the pathway related to SO-deficiency). This pathway was inspired by Chapter 12 of the book of Blau (ISBN 3642403360 (978-3642403361)).
last edited


DeSl, Egonw, Josienlandman, IreneHemel, MaintBot, and Finterly

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Homo sapiens


Inborn Errors of Metabolism (IEM) Pathways Rare Diseases


Disease Ontology: molybdenum cofactor deficiency type B xanthinuria molybdenum cofactor deficiency type C molybdenum cofactor deficiency

Pathway Ontology: molybdenum cofactor biosynthetic pathway molybdenum cofactor deficiency pathway xanthinuria type I pathway xanthinuria type II pathway altered molybdenum cofactor biosynthetic pathway


Label Type Compact Identifier
Sulfite oxidase Protein uniprot:P51687
cPMP Metabolite chebi:52994
Gephyrin(G domain) Protein uniprot:Q9NQX3
Moco Metabolite chebi:71306
R=N-OH Metabolite None
MOCS1A Protein ensembl:ENSG00000124615
MPT Metabolite chebi:44074
GTP Metabolite chebi:37565
Sulfite Metabolite chebi:26823
Xanthine Metabolite chebi:17712
MPT-AMP Metabolite chebi:62728
Amidoxime reducingcomponent Protein uniprot:Q969Z3
Xanthine oxidase Protein uniprot:P47989
Aldehyde oxidase Protein uniprot:Q06278
Sulfate Metabolite hmdb:HMDB0001448
R-NH Metabolite None
urate Metabolite chebi:17775
R-H Metabolite None
R-OH Metabolite chebi:17478
Sulfite oxidase Protein uniprot:P51687
Moco Metabolite chebi:21437
Amidoxime reducingcomponent Protein uniprot:Q969Z3
Moco Metabolite chebi:21437
Xanthine oxidase Protein uniprot:P47989
Moco Metabolite chebi:21437
Aldehyde oxidase Protein uniprot:Q06278
Moco Metabolite chebi:21437
MOCS1AB Protein None
MOCS2A Protein uniprot:O96007
MOCS2B Protein uniprot:O96033
Gephyrin(E domain) Protein uniprot:Q9NQX3


  1. Llamas A, Mendel RR, Schwarz G. Synthesis of adenylated molybdopterin: an essential step for molybdenum insertion. J Biol Chem. 2004 Dec 31;279(53):55241–6. PubMed Europe PMC Scholia
  2. Schwarz G. Molybdenum cofactor biosynthesis and deficiency. Cell Mol Life Sci. 2005 Dec;62(23):2792–810. PubMed Europe PMC Scholia
  3. PubMed Europe PMC Scholia