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Details on Person UniProt:Q9HCC0 MCCC2
| Class:Id | ReferenceGeneProduct:59059 |
|---|---|
| _chainChangeLog | transit peptide:1-22 added on Sat February 7 2015;chain:23-563 added on Sat February 7 2015 |
| _displayName | UniProt:Q9HCC0 MCCC2 |
| _timestamp | 2025-05-21 21:31:16 |
| chain | transit peptide:1-22 chain:23-563 |
| checksum | 8E3D401AF52DC7D2 |
| comment | FUNCTION Carboxyltransferase 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(+)BIOPHYSICOCHEMICAL PROPERTIES kcat is 4.0 sec(-1).PATHWAY 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.INTERACTION The disease is caused by variants affecting the gene represented in this entry.SIMILARITY Belongs to the AccD/PCCB family. |
| description | recommendedName: Methylcrotonoyl-CoA carboxylase beta chain, mitochondrial shortName: MCCase subunit beta ecNumber evidence="12"6.4.1.4 alternativeName: 3-methylcrotonyl-CoA carboxylase 2 alternativeName: 3-methylcrotonyl-CoA carboxylase non-biotin-containing subunit alternativeName: 3-methylcrotonyl-CoA:carbon dioxide ligase subunit beta |
| geneName | MCCC2 MCCB |
| identifier | Q9HCC0 |
| isSequenceChanged | FALSE |
| keyword | 3D-structure Acetylation Alternative splicing ATP-binding 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:9862192] Weiser, Joel, 2024-02-26 [InstanceEdit:9917590] Weiser, Joel, 2024-08-09 [InstanceEdit:9926675] Weiser, Joel, 2024-11-03 [InstanceEdit:9939033] Weiser, Joel, 2025-02-21 [InstanceEdit:9948485] Weiser, Joel, 2025-05-21 |
| name | MCCC2 |
| referenceDatabase | [ReferenceDatabase:2] UniProt |
| referenceGene | [ReferenceDNASequence:9001265] ENSEMBL:ENSG00000131844 MCCC2 [Homo sapiens] |
| secondaryIdentifier | MCCB_HUMAN A6NIY9 Q96C27 Q9Y4L7 |
| sequenceLength | 563 |
| species | [Species:48887] Homo sapiens |
| (isoformParent) | [ReferenceIsoform:150125] UniProt:Q9HCC0-2 MCCC2 [Homo sapiens] [ReferenceIsoform:414419] UniProt:Q9HCC0-1 MCCC2 [Homo sapiens] |
| (referenceEntity) | [EntityWithAccessionedSequence:70769] MCCC2 [mitochondrial matrix] [Homo sapiens] [EntityWithAccessionedSequence:3323134] MCCC2 [cytosol] [Homo sapiens] [EntityWithAccessionedSequence:9909439] S173L MCCC2 [mitochondrial matrix] [Homo sapiens] [EntityWithAccessionedSequence:9909449] A456V MCCC2 [mitochondrial matrix] [Homo sapiens] [EntityWithAccessionedSequence:9909455] V339M MCCC2 [mitochondrial matrix] [Homo sapiens] [EntityWithAccessionedSequence:9909463] E99Q MCCC2 [mitochondrial matrix] [Homo sapiens] [EntityWithAccessionedSequence:9909469] H190Y MCCC2 [mitochondrial matrix] [Homo sapiens] [EntityWithAccessionedSequence:9909470] G352R MCCC2 [mitochondrial matrix] [Homo sapiens] |
| (referenceSequence) | [ReplacedResidue:9909437] L-glutamic acid 99 replaced with L-glutamine [ReplacedResidue:9909444] glycine 352 replaced with L-arginine [ReplacedResidue:9909446] L-histidine 190 replaced with L-tyrosine [ReplacedResidue:9909453] L-valine 339 replaced with L-methionine [ReplacedResidue:9909460] L-serine 173 replaced with L-leucine [ReplacedResidue:9909464] L-alanine 456 replaced with L-valine |
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No pathways have been reviewed or authored by UniProt:Q9HCC0 MCCC2 (59059)
