MOCS1 Antibody – Rabbit Polyclonal

Function: Isoform MOCS1A and isoform MOCS1B probably form a complex that catalyzes the conversion of 5'-GTP to cyclic pyranopterin monophosphate (cPMP or molybdopterin precursor Z). Ref.8

Catalytic activity: GTP = cyclic pyranopterin phosphate + diphosphate. Ref.8

Cofactor: Binds 2 4Fe-4S clusters. Binds 1 4Fe-4S cluster coordinated with 3 cysteines and an exchangeable S-adenosyl-L-methionine and 1 4Fe-4S cluster coordinated with 3 cysteines and the GTP-derived substrate. Ref.10

Pathway: Cofactor biosynthesis; molybdopterin biosynthesis.

Subunit structure: Isoform MOCS1A and isoform MOCS1B probably form a heterooligomer

Probable.

Tissue specificity: Isoform MOCS1A and isoform 2 are widely expressed. Ref.1 Ref.9

Post-translational modification: Isoform MOCS1A, isoform 2 and isoform 3 are probably thiocarboxylated at their C-terminus. Thiocarboxylation probably plays a central role in molybdenum cofactor biosynthesis, since mutagenesis of the last 2 Gly residues of isoform MOCS1A abolishes the catalytic activity of the enzyme. Thiocarboxylation is absent in isoform MOCS1B, which lacks the C-terminal Gly residue.

Involvement in disease: Molybdenum cofactor deficiency type A (MOCOD type A) [MIM:252150]: Autosomal recessive disease which leads to the pleiotropic loss of all molybdoenzyme activities and is characterized by severe neurological damage, neonatal seizures and early childhood death.Note: The disease is caused by mutations affecting the gene represented in this entry.

Miscellaneous: The MOCS1 locus has initially been reported to produce MOCS1A and MOCS1B from non-overlapping reading frames within a bicistronic transcript. However, only isoform MOCS1A seems to be translated from the bicistronic transcript. Isoform MOCS1B seems to be translated from a monocistronic mRNA that is derived by alternative splicing.

Sequence similarities: In the C-terminal section; belongs to the MoaC family.In the N-terminal section; belongs to the MoaA/NifB/PqqE family.

Sequence caution: The sequence AAB87524.1 differs from that shown. Reason: Alternative splicing in the MOCS1A-MOCS1B joining region.The sequence AAS00489.1 differs from that shown. Reason: Erroneous initiation. The sequence CAC44526.1 differs from that shown. Reason: Alternative splicing in the MOCS1A-MOCS1B joining region.The sequence CAI20007.1 differs from that shown. Reason: Erroneous gene model prediction. The sequence CAI20012.1 differs from that shown. Reason: Erroneous gene model prediction. The sequence CAI20013.1 differs from that shown. Reason: Erroneous gene model prediction.