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Details on Person UniProt:Q9NZB8-1 MOCS1
| Class:Id | ReferenceIsoform:414940 |
|---|---|
| _chainChangeLog | chain:1-636 added on Fri February 6 2015;chain:1-636 for 414940 removed on Fri Feb 20 2026;transit peptide:1-21 for 414940 added on Fri Feb 20 2026;chain:22-636 for 414940 added on Fri Feb 20 2026;propeptide:22-448 for 414940 added on Fri Feb 20 2026 |
| _displayName | UniProt:Q9NZB8-1 MOCS1 |
| _timestamp | 2026-02-20 21:51:14 |
| chain | transit peptide:1-21 chain:22-636 propeptide:22-448 |
| checksum | 6774A563BAC42120 |
| comment | FUNCTION Isoform MOCS1A and isoform MOCS1B probably form a complex that catalyzes the conversion of 5'-GTP to cyclic pyranopterin monophosphate (cPMP) (PubMed:11891227, PubMed:23627491, PubMed:29368224, PubMed:31996372). MOCS1A catalyzes the cyclization of GTP to (8S)-3',8-cyclo-7,8-dihydroguanosine 5'-triphosphate and MOCS1B catalyzes the subsequent conversion of (8S)-3',8-cyclo-7,8-dihydroguanosine 5'-triphosphate to cPMP (PubMed:11891227, PubMed:23627491, PubMed:29368224, PubMed:31996372).FUNCTION Has very weak catalytic activity.CATALYTIC ACTIVITY GTP + AH2 + S-adenosyl-L-methionine = (8S)-3',8-cyclo-7,8-dihydroguanosine 5'-triphosphate + 5'-deoxyadenosine + L-methionine + A + H(+)CATALYTIC ACTIVITY GTP + AH2 + S-adenosyl-L-methionine = (8S)-3',8-cyclo-7,8-dihydroguanosine 5'-triphosphate + 5'-deoxyadenosine + L-methionine + A + H(+)CATALYTIC ACTIVITY GTP + AH2 + S-adenosyl-L-methionine = (8S)-3',8-cyclo-7,8-dihydroguanosine 5'-triphosphate + 5'-deoxyadenosine + L-methionine + A + H(+)CATALYTIC ACTIVITY (8S)-3',8-cyclo-7,8-dihydroguanosine 5'-triphosphate = cyclic pyranopterin phosphate + diphosphateCOFACTOR 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.BIOPHYSICOCHEMICAL PROPERTIES kcat is 0.092 min(-1) for (8S)-3',8-cyclo-7,8-dihydroguanosine 5'-triphosphate.PATHWAY Cofactor biosynthesis; molybdopterin biosynthesis.SUBUNIT Isoform MOCS1A and isoform MOCS1B probably form a heterooligomer.SUBCELLULAR LOCATION Found in aggregates not co-localized with mitochondria.SUBCELLULAR LOCATION So far, the different types of MOCS1A and MOCS1B isoforms have been investigated independently and several combinations might be possible.TISSUE SPECIFICITY Isoform MOCS1A and isoform 2 are widely expressed.DISEASE The disease is caused by variants affecting the gene represented in this entry.MISCELLANEOUS The MOCS1 transcript is alternatively spliced resulting in production of alternative MOCS1A and MOCS1B products (PubMed:10917590, PubMed:11891227, PubMed:31996372). Type I splicing results in bicistronic transcripts with two open reading frames, of which only the first, MOCS1A, is translated due to a stop codon in exon 9 (PubMed:10917590, PubMed:11891227, PubMed:31996372). Alternative splicing of exon 9 leads to production of type II or III transcripts (where exon 9 is partially or fully removed) and where MOCS1B is translated as a fusion protein together with catalytically inactive MOCS1A; inactive MOCS1A is subsequently proteolytically cleaved during mitochondrial import and it is not known if it has a functional role (PubMed:10917590, PubMed:11891227, PubMed:31996372).MISCELLANEOUS Alternative splicing of the N-terminal exon 1 cassettes a-d appears to impart different properties to the protein; exon 1a targets MOCS1A to the mitochondrion whereas exon 1b at the N-terminus remains in the cytosol, exon 1c might stabilize MOCS1A, and exon 1d is essential for MOCS1A catalytic activity (PubMed:31996372). Exon 1a seems dispensable for targeting MOCS1B to the mitochondrion due to the presence of a linker region separating MOCS1A and MOCS1B that appears to facilitate mitochondrial import (PubMed:31996372).MISCELLANEOUS May be produced at very low levels due to a premature stop codon in the mRNA, leading to nonsense-mediated mRNA decay.MISCELLANEOUS Multidomain protein with inactive MOCS1A and active MOCS1B.SIMILARITY In the C-terminal section; belongs to the MoaC family.SIMILARITY In the N-terminal section; belongs to the radical SAM superfamily. MoaA family.CAUTION The C-terminus of MOCS1A was previously believed to be thiocarboxylated, but it is now known not to be the case.SEQUENCE CAUTION Alternative splicing in the MOCS1A-MOCS1B joining region.SEQUENCE CAUTION Alternative splicing in the MOCS1A-MOCS1B joining region. |
| created | [InstanceEdit:400710] Schmidt, EE, 2009-03-25 05:33:35 |
| description | recommendedName: Molybdenum cofactor biosynthesis protein 1 alternativeName: Cell migration-inducing gene 11 protein alternativeName: Molybdenum cofactor synthesis-step 1 protein A-B domain recommendedName: fullName evidence="42"GTP 3',8-cyclase ecNumber evidence="25"4.1.99.22 alternativeName: fullName evidence="42"MOCS1A alternativeName: Molybdenum cofactor biosynthesis protein A /domain domain recommendedName: fullName evidence="42"Cyclic pyranopterin monophosphate synthase ecNumber evidence="20"4.6.1.17 alternativeName: fullName evidence="42"MOCS1B alternativeName: Molybdenum cofactor biosynthesis protein C /domain |
| geneName | MOCS1 MIG11 |
| identifier | Q9NZB8 |
| isoformParent | |
| isSequenceChanged | FALSE |
| keyword | 4Fe-4S Acetylation Alternative splicing Cytoplasm Disease variant GTP-binding Iron Iron-sulfur Lyase Metal-binding Mitochondrion Molybdenum cofactor biosynthesis Nucleotide-binding Phosphoprotein Proteomics identification Reference proteome S-adenosyl-L-methionine Transit peptide |
| modified | [InstanceEdit:9836292] Weiser, Joel, 2023-05-25 [InstanceEdit:9852000] Weiser, Joel, 2023-11-03 [InstanceEdit:9926675] Weiser, Joel, 2024-11-03 [InstanceEdit:9939033] Weiser, Joel, 2025-02-21 [InstanceEdit:9983091] Weiser, Joel, 2026-02-20 |
| name | MOCS1 |
| referenceDatabase | [ReferenceDatabase:2] UniProt |
| referenceGene | [ReferenceDNASequence:8999003] ENSEMBL:ENSG00000124615 MOCS1 [Homo sapiens] |
| secondaryIdentifier | MOCS1_HUMAN B3KPT7 B4DTP1 O14940 O14941 O75710 Q5J7W0 Q5TCE1 Q5TCE2 Q5TCE6 Q5TCE9 Q5TCF0 Q5TCF1 Q8N418 Q9NZB7 Q9UEM1 |
| sequenceLength | 636 |
| species | [Species:48887] Homo sapiens |
| variantIdentifier | Q9NZB8-1 |
| (referenceEntity) | [EntityWithAccessionedSequence:947547] MOCS1-1 [cytosol] [Homo sapiens] |
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No pathways have been reviewed or authored by UniProt:Q9NZB8-1 MOCS1 (414940)
