UniProt:Q9HCC0 MCCC2

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.
databaseName UniProt
dbId 59059
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
displayName UniProt:Q9HCC0 MCCC2
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
moleculeType Protein
name
  • MCCC2
otherIdentifier
  • 11729236_at
  • 11729237_a_at
  • 1556345_PM_s_at
  • 1556345_s_at
  • 1560033_PM_at
  • 1560033_at
  • 16985911
  • 209623_PM_at
  • 209623_at
  • 209624_PM_s_at
  • 209624_s_at
  • 233231_PM_at
  • 233231_at
  • 2814641
  • 2814643
  • 2814644
  • 2814647
  • 2814648
  • 2814649
  • 2814650
  • 2814651
  • 2814652
  • 2814655
  • 2814663
  • 2814664
  • 2814665
  • 2814666
  • 2814667
  • 2814668
  • 2814669
  • 2814670
  • 2814671
  • 2814672
  • 2814673
  • 2814674
  • 2814675
  • 2814676
  • 2814677
  • 2814678
  • 2814679
  • 2814680
  • 2814681
  • 2814682
  • 2814687
  • 2814688
  • 2814689
  • 2814690
  • 2814691
  • 2814692
  • 36432_at
  • 43695_at
  • 64087
  • 72262_at
  • 8106068
  • 8177601
  • 88457_at
  • A_14_P111147
  • A_14_P122432
  • A_23_P18887
  • GE56317
  • GE88197
  • GE905935
  • GO:0000166
  • GO:0004485
  • GO:0005515
  • GO:0005524
  • GO:0005739
  • GO:0005759
  • GO:0005829
  • GO:0006520
  • GO:0006552
  • GO:0006790
  • GO:0009083
  • GO:0015936
  • GO:0016874
  • GO:0055086
  • GO:1905202
  • HMNXSV003034390
  • Hs.167531.0.S2_3p_at
  • Hs.272240.0.S1_3p_at
  • Hs2.288415.1.S1_3p_at
  • Hs2.407347.1.S1_3p_s_at
  • PH_hs_0019391
  • PH_hs_0027271
  • PH_hs_0038535
  • TC05000334.hg
  • g10934058_3p_a_at
schemaClass ReferenceGeneProduct
secondaryIdentifier
  • MCCB_HUMAN
  • A6NIY9
  • Q96C27
  • Q9Y4L7
sequenceLength 563
stId uniprot:Q9HCC0
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