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)). d1b dcc AKA metal-binding pterin AKA adenylylated MPT dc8 c93 dc8 dc8 xanthinuria DOID:0060236 Disease molybdenum cofactor deficiency pathway PW:0001817 Pathway Ontology xanthinuria type I pathway PW:0001591 Pathway Ontology molybdenum cofactor deficiency type B DOID:0111163 Disease molybdenum cofactor deficiency DOID:0111165 Disease 16261263 PubMed Molybdenum cofactor biosynthesis and deficiency. Cell Mol Life Sci 2005 Schwarz G 10.1101/429183 DOI Alternative splicing of bicistronic MOCS1 defines a novel mitochondrial protein maturation mechanism https://doi.org/10.1101/429183 2018 Simon Julius Mayr Juliane Roeper Guenter Schwarz 15504727 PubMed Synthesis of adenylated molybdopterin: an essential step for molybdenum insertion. J Biol Chem 2004 Llamas A Mendel RR Schwarz G xanthinuria type II pathway PW:0001592 Pathway Ontology altered molybdenum cofactor biosynthetic pathway PW:0000428 Pathway Ontology molybdenum cofactor deficiency type C DOID:0111166 Disease 9783642403361 ISBN 'Physician's Guide to the Diagnosis, Treatment, and Follow-Up of Inherited Metabolic Diseases' Springer-Verlag Berlin Heidelberg 2014 Nenad Blau Marinus Duran K Michael Gibson Carlo Dionisi-Vici molybdenum cofactor biosynthetic pathway PW:0000427 Pathway Ontology