DISEASES

Disease-gene associations mined from literature

Literature associating MOCS3 and encephalomalacia

MOCS3 [ENSP00000244051]

Adenylyltransferase and sulfurtransferase MOCS3; 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. ; In the N-terminal section; belongs to the HesA/MoeB/ThiF family. UBA4 subfamily.

Synonyms:  MOCS3,  MOCS3p,  hMOCS3,  O95396,  O95396p ...

Linkouts:  STRING  Pharos  UniProt  OMIM