Aliases for MOCS3 Gene
- Molybdenum Cofactor Synthesis 3 2 3 5
- UBA4 2 3 4
- Ubiquitin-Like Modifier Activating Enzyme 4 2 3
- Molybdenum Cofactor Synthesis Protein 3 3 4
- Molybdopterin Synthase Sulfurylase 3 4
- MPT Synthase Sulfurylase 3 4
- Ubiquitin-Activating Enzyme E1 Homolog (Yeast) 2
- UBA4, Ubiquitin-Activating Enzyme E1 Homolog 3
External Ids for MOCS3 Gene
Previous GeneCards Identifiers for MOCS3 Gene
Molybdenum cofactor (MoCo) is necessary for the function of all molybdoenzymes. The protein encoded by this gene adenylates and activates molybdopterin synthase, an enzyme required for biosynthesis of MoCo. This gene contains no introns. A pseudogene of this gene is present on chromosome 14. [provided by RefSeq, Nov 2012]
GeneCards Summary for MOCS3 Gene
MOCS3 (Molybdenum Cofactor Synthesis 3) is a Protein Coding gene. Diseases associated with MOCS3 include molybdenum cofactor deficiency. Among its related pathways are Metabolism and Metabolism of water-soluble vitamins and cofactors. GO annotations related to this gene include nucleotidyltransferase activity and sulfurtransferase activity. An important paralog of this gene is UBA5.
UniProtKB/Swiss-Prot for MOCS3 Gene
Plays a central role in 2-thiolation of mcm(5)S(2)U at tRNA wobble positions of cytosolic tRNA(Lys), tRNA(Glu) and tRNA(Gln). Also essential during biosynthesis of the molybdenum cofactor. Acts by mediating the C-terminal thiocarboxylation of sulfur carriers URM1 and MOCS2A. Its N-terminus first activates URM1 and MOCS2A as acyl-adenylates (-COAMP), then the persulfide sulfur on the catalytic cysteine is transferred to URM1 and MOCS2A to form thiocarboxylation (-COSH) of their C-terminus. The reaction probably involves hydrogen sulfide that is generated from the persulfide intermediate and that acts as nucleophile towards URM1 and MOCS2A. Subsequently, a transient disulfide bond is formed. Does not use thiosulfate as sulfur donor; NFS1 probably acting as a sulfur donor for thiocarboxylation reactions.