precursor Z + 2 [molybdopterin-synthase sulfur-carrier protein]-Gly-NH-CH2-C(O)SH + H2O molybdopterin + 2 molybdopterin-synthase sulfur-carrier protein
Molybdopterin synthase is heterodimeric and coded for by the MOCS2 gene.[3] Genetic deficiencies of enzymes such as MPT synthase, which are involved in MoCo biosynthesis, lead to MoCo deficiency, a rare disease that results in severe neurological abnormalities.[4][5][6][7]
^Daniels JN, Wuebbens MM, Rajagopalan KV, Schindelin H (January 2008). "Crystal structure of a molybdopterin synthase-precursor Z complex: insight into its sulfur transfer mechanism and its role in molybdenum cofactor deficiency". Biochemistry. 47 (2): 615–26. doi:10.1021/bi701734g. PMID18092812.
^Rudolph, Michael J. and Wuebbens, Margot M. and Rajagopalan, K. V. and Schindelin, Hermann (2001). "Crystal structure of molybdopterin synthase and its evolutionary relationship to ubiquitin activation". Nature Structural Biology. 8 (1): 42–46. doi:10.1038/83034. PMID11135669. S2CID10494830.{{cite journal}}: CS1 maint: multiple names: authors list (link)
^Reiss J (2000). "Genetics of molybdenum cofactor deficiency". Human Genetics. 106 (2): 157–163. doi:10.1007/s004390051023 (inactive 2024-03-28). PMID10746556.{{cite journal}}: CS1 maint: DOI inactive as of March 2024 (link)