Molybdenum cofactor (Moco) biosynthesis (WP4507)

Homo sapiens

Open in new tab Open in NDEx Open in Newt (SBGN)
Download PNG Download SVG Download JSON Download GPML Learn more about Downloads
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 [https://www.wikipathways.org/index.php/Pathway:WP4504] for the pathway related to SO-deficiency). This pathway was inspired by Chapter 12 of the book of Blau (ISBN 3642403360 (978-3642403361)).

Authors

Denise Slenter , Egon Willighagen , Josien Landman , Irene Hemel , Finterly Hu , and Eric Weitz

Activity

last edited

Discuss this pathway

Check for ongoing discussions or start your own.

Cited In

Are you planning to include this pathway in your next publication? See How to Cite and add a link here to your paper once it's online.

Organisms

Homo sapiens

Communities

Inherited Metabolic Disorders (IMD) Pathways Rare Diseases

Annotations

Disease Ontology

xanthinuria molybdenum cofactor deficiency type B molybdenum cofactor deficiency molybdenum cofactor deficiency type C

Pathway Ontology

molybdenum cofactor deficiency pathway xanthinuria type I pathway xanthinuria type II pathway altered molybdenum cofactor biosynthetic pathway molybdenum cofactor biosynthetic pathway

Participants
Query Drugst.One

Label Type Compact URI Comment
cPMP Metabolite chebi:52994
Moco Metabolite chebi:71306
MPT Metabolite chebi:44074 AKA metal-binding pterin
GTP Metabolite chebi:37565
Sulfite Metabolite chebi:26823
Xanthine Metabolite chebi:17712
MPT-AMP Metabolite chebi:62728 AKA adenylylated MPT
Sulfate Metabolite hmdb:HMDB0001448
urate Metabolite chebi:17775
R-OH Metabolite chebi:17478
Moco Metabolite chebi:21437
Sulfite oxidase Protein uniprot:P51687
Gephyrin(G domain) Protein uniprot:Q9NQX3
MOCS1A Protein ensembl:ENSG00000124615
Amidoxime reducingcomponent Protein uniprot:Q969Z3
Xanthine oxidase Protein uniprot:P47989
Aldehyde oxidase Protein uniprot:Q06278
MOCS2A Protein uniprot:O96007
MOCS2B Protein uniprot:O96033
Gephyrin(E domain) Protein uniprot:Q9NQX3

References

  1. Alternative splicing of bicistronic MOCS1 defines a novel mitochondrial protein maturation mechanism [Internet]. Mayr SJ, Röper J, Schwarz G. Cold Spring Harbor Laboratory; 2018. Available from: http://dx.doi.org/10.1101/429183 DOI Scholia
  2. Physician’s Guide to the Diagnosis, Treatment, and Follow-Up of Inherited Metabolic Diseases [Internet]. Blau N, Duran M, Gibson KM, Dionisi-Vici C. Springer; 2014. 0 p. Available from: https://books.google.com/books/about/Physician_s_Guide_to_the_Diagnosis_Treat.html?hl=&id=wJRBnwEACAAJ OpenLibrary Worldcat
  3. Synthesis of adenylated molybdopterin: an essential step for molybdenum insertion. Llamas A, Mendel RR, Schwarz G. J Biol Chem. 2004 Dec 31;279(53):55241–6. PubMed Europe PMC Scholia
  4. Molybdenum cofactor biosynthesis and deficiency. Schwarz G. Cell Mol Life Sci. 2005 Dec;62(23):2792–810. PubMed Europe PMC Scholia