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Details on Person UniProt:Q96RQ3 MCCC1
| Class:Id | ReferenceGeneProduct:59057 |
|---|---|
| _chainChangeLog | transit peptide:1-41 added on Fri February 6 2015;chain:42-725 added on Fri February 6 2015 |
| _displayName | UniProt:Q96RQ3 MCCC1 |
| _timestamp | 2025-05-21 21:31:15 |
| chain | transit peptide:1-41 chain:42-725 |
| checksum | B84AD23806035A40 |
| comment | FUNCTION Biotin-attachment subunit of the 3-methylcrotonyl-CoA carboxylase, an enzyme that catalyzes the conversion of 3-methylcrotonyl-CoA to 3-methylglutaconyl-CoA, a critical step for leucine and isovaleric acid catabolism.CATALYTIC ACTIVITY 3-methylbut-2-enoyl-CoA + hydrogencarbonate + ATP = 3-methyl-(2E)-glutaconyl-CoA + ADP + phosphate + H(+)COFACTOR Amino-acid degradation; L-leucine degradation; (S)-3-hydroxy-3-methylglutaryl-CoA from 3-isovaleryl-CoA: step 2/3.SUBUNIT Probably a dodecamer composed of six biotin-containing alpha subunits (MCCC1) and six beta (MCCC2) subunits (PubMed:17360195). Interacts (via the biotin carboxylation domain) with SIRT4 (PubMed:23438705).INTERACTION Acetylated.DISEASE The disease is caused by variants affecting the gene represented in this entry.SEQUENCE CAUTION Extended N-terminus. |
| description | recommendedName: Methylcrotonoyl-CoA carboxylase subunit alpha, mitochondrial shortName: MCCase subunit alpha ecNumber evidence="12 22"6.4.1.4 alternativeName: 3-methylcrotonyl-CoA carboxylase 1 alternativeName: 3-methylcrotonyl-CoA carboxylase biotin-containing subunit alternativeName: 3-methylcrotonyl-CoA:carbon dioxide ligase subunit alpha |
| geneName | MCCC1 MCCA |
| identifier | Q96RQ3 |
| isSequenceChanged | FALSE |
| keyword | 3D-structure Acetylation ATP-binding Biotin Direct protein sequencing Disease variant Ligase Mitochondrion Nucleotide-binding Proteomics identification Reference proteome 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:9948485] Weiser, Joel, 2025-05-21 |
| name | MCCC1 |
| referenceDatabase | [ReferenceDatabase:2] UniProt |
| referenceGene | [ReferenceDNASequence:8997266] ENSEMBL:ENSG00000078070 MCCC1 [Homo sapiens] |
| secondaryIdentifier | MCCA_HUMAN Q59ES4 Q9H959 Q9NS97 |
| sequenceLength | 725 |
| species | [Species:48887] Homo sapiens |
| (referenceEntity) | [EntityWithAccessionedSequence:70748] Btn-MCCC1 [mitochondrial matrix] [Homo sapiens] [EntityWithAccessionedSequence:2993803] MCCC1 [mitochondrial matrix] [Homo sapiens] [EntityWithAccessionedSequence:3323154] Btn-MCCC1 [cytosol] [Homo sapiens] [EntityWithAccessionedSequence:3323164] MCCC1 [cytosol] [Homo sapiens] [EntityWithAccessionedSequence:9908652] D532H Btn-MCCC1 [mitochondrial matrix] [Homo sapiens] [EntityWithAccessionedSequence:9908657] I460M Btn-MCCC1 [mitochondrial matrix] [Homo sapiens] [EntityWithAccessionedSequence:9908666] L437P Btn-MCCC1 [mitochondrial matrix] [Homo sapiens] [EntityWithAccessionedSequence:9908669] M325R Btn-MCCC1 [mitochondrial matrix] [Homo sapiens] [EntityWithAccessionedSequence:9908676] R385S Btn-MCCC1 [mitochondrial matrix] [Homo sapiens] [EntityWithAccessionedSequence:9908680] S535F Btn-MCCC1 [mitochondrial matrix] [Homo sapiens] |
| (referenceSequence) | [ModifiedResidue:508303] N6-biotinyl-L-lysine at 681 [ReplacedResidue:9908653] L-aspartic acid 532 replaced with L-histidine [ReplacedResidue:9908656] L-isoleucine 460 replaced with L-methionine [ReplacedResidue:9908660] L-arginine 385 replaced with L-serine [ReplacedResidue:9908663] L-leucine 437 replaced with L-proline [ReplacedResidue:9908664] L-methionine 325 replaced with L-arginine [ReplacedResidue:9908672] L-serine 535 replaced with L-phenylalanine [NonsenseMutation:9908674] Nonsense mutation at L-valine 694 |
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No pathways have been reviewed or authored by UniProt:Q96RQ3 MCCC1 (59057)
