| Molybdopterin synthase | |||||||||
|---|---|---|---|---|---|---|---|---|---|
| Identifiers | |||||||||
| EC no. | 2.8.1.12 | ||||||||
| Databases | |||||||||
| IntEnz | IntEnz view | ||||||||
| BRENDA | BRENDA entry | ||||||||
| ExPASy | NiceZyme view | ||||||||
| KEGG | KEGG entry | ||||||||
| MetaCyc | metabolic pathway | ||||||||
| PRIAM | profile | ||||||||
| PDB structures | RCSB PDB PDBe PDBsum | ||||||||
| |||||||||
Molybdopterin synthase (EC 2.8.1.12, MPT synthase) is an enzyme required to synthesize molybdopterin (MPT) from precursor Z (now known as cyclic pyranopterin monophosphate).[1][2] Molydopterin is subsequently complexed with molybdenum to form molybdenum cofactor (MoCo). MPT synthase catalyses the following chemical reaction:
- 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. PMID 18092812.
- ^ Wuebbens MM, Rajagopalan KV (April 2003). "Mechanistic and mutational studies of Escherichia coli molybdopterin synthase clarify the final step of molybdopterin biosynthesis". The Journal of Biological Chemistry. 278 (16): 14523–32. doi:10.1074/jbc.m300453200. PMID 12571226.
- ^ Sloan J, Kinghorn JR, Unkles SE (February 1999). "The two subunits of human molybdopterin synthase: evidence for a bicistronic messenger RNA with overlapping reading frames". Nucleic Acids Research. 27 (3): 854–8. doi:10.1093/nar/27.3.854. PMC 148257. PMID 9889283.
- ^ 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. PMID 11135669. S2CID 10494830.
{{cite journal}}: CS1 maint: multiple names: authors list (link) - ^ Silke Leimkühler, Andrea Freuer, Jose ́ Angel Santamaria Araujo, K. V. Rajagopalan, and Ralf R. Mendel (2003). "Mechanistic Studies of Human Molybdopterin Synthase Reaction and Characterization of Mutants Identified in Group B Patients of Molybdenum Cofactor Deficiency". Journal of Biological Chemistry. 278 (28): 26127–26134. doi:10.1074/jbc.M303092200. PMID 12732628.
{{cite journal}}: CS1 maint: multiple names: authors list (link) - ^ Stallmeyer, B., Schwarz, G., Schulze, J., Nerlich, A., Reiss, J., Kirsch, J., Mendel, R. R. (1999). "The neurotransmitter receptor-anchoring protein gephyrin reconstitutes molybdenum cofactor biosynthesis in bacteria, plants, and mammalian cells". Proceedings of the National Academy of Sciences of the United States of America. 96 (4): 1333–1338. Bibcode:1999PNAS...96.1333S. doi:10.1073/pnas.96.4.1333. PMC 15463. PMID 9990024.
{{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). PMID 10746556.
{{cite journal}}: CS1 maint: DOI inactive as of March 2024 (link)