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)). d1bdccAKA metal-binding pterinAKA adenylylated MPTdc8c93dc8dc8xanthinuriaDOID:0060236Diseasemolybdenum cofactor deficiency pathwayPW:0001817Pathway Ontologyxanthinuria type I pathwayPW:0001591Pathway Ontologymolybdenum cofactor deficiency type BDOID:0111163Diseasemolybdenum cofactor deficiencyDOID:0111165Disease16261263PubMedMolybdenum cofactor biosynthesis and deficiency.Cell Mol Life Sci2005Schwarz G10.1101/429183DOIAlternative splicing of bicistronic MOCS1 defines a novel mitochondrial protein maturation mechanismhttps://doi.org/10.1101/4291832018Simon Julius MayrJuliane RoeperGuenter Schwarz15504727PubMedSynthesis of adenylated molybdopterin: an essential step for molybdenum insertion.J Biol Chem2004Llamas AMendel RRSchwarz Gxanthinuria type II pathwayPW:0001592Pathway Ontologyaltered molybdenum cofactor biosynthetic pathwayPW:0000428Pathway Ontologymolybdenum cofactor deficiency type CDOID:0111166Disease9783642403361ISBN'Physician's Guide to the Diagnosis, Treatment, and Follow-Up of Inherited Metabolic Diseases'Springer-Verlag Berlin Heidelberg2014Nenad BlauMarinus DuranK Michael GibsonCarlo Dionisi-Vicimolybdenum cofactor biosynthetic pathwayPW:0000427Pathway Ontology