molybdenum cofactor biosynthesis protein 1; cell migration-inducing gene 11 protein; molybdenum cofactor synthesis-step 1 protein A-B; includes: molybdenum cofactor biosynthesis protein A; includes: molybdenum cofactor biosynthesis protein C (MOCS1)
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Function
- isoform MOCS1A & isoform MOCS1B probably form a complex that catalyzes the conversion of a guanosine derivative to precursor Z during molybdenum cofactor biosynthesis cofactor biosynthesis; molybdopterin biosynthesis
- isoform MOCS1A, isoform 2 & isoform 3 are probably thiocarboxylated at their C-terminus
- thiocarboxylation probably plays a role in molybdenum cofactor biosynthesis, since mutagenesis of the last 2 Gly of isoform MOCS1A abolishes catalytic activity of the enzyme
- thiocarboxylation is absent in isoform MOCS1B, which lacks the C-terminal Gly
- fosdenopterin cofactor
- binds 2 4Fe-4S clusters
- binds 1 4Fe-4S cluster coordinated with 3 Cys & an exchangeable S-adenosyl-L-methionine & 1 4Fe-4S cluster coordinated with 3 Cys & the GTP-derived substrate
- isoform MOCS1A & isoform MOCS1B probably form a heterooligomer (probable)
Structure
- in the C-terminal section; belongs to the moaC family
- in the N-terminal section; belongs to the moaA/nifB/pqqE family
Alternative splicing
named isoforms=8
Expression
Pathology
- defects in MOCS1 are the cause of molybdenum cofactor deficiency type A
Notes
- the MOCS1 locus has initially been reported to produce MOCS1A & 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