UniProt:P26440 IVD

chain
  • transit peptide:1-32
  • chain:33-426
checksum D744DA85752BF307
comment
  • FUNCTION Catalyzes the conversion of isovaleryl-CoA/3-methylbutanoyl-CoA to 3-methylbut-2-enoyl-CoA as an intermediate step in the leucine (Leu) catabolic pathway (PubMed:7640268). To a lesser extent, is also able to catalyze the oxidation of other saturated short-chain acyl-CoA thioesters as pentanoyl-CoA, hexenoyl-CoA and butenoyl-CoA (PubMed:7640268).CATALYTIC ACTIVITY 3-methylbutanoyl-CoA + oxidized [electron-transfer flavoprotein] + H(+) = 3-methylbut-2-enoyl-CoA + reduced [electron-transfer flavoprotein]CATALYTIC ACTIVITY pentanoyl-CoA + oxidized [electron-transfer flavoprotein] + H(+) = (2E)-pentenoyl-CoA + reduced [electron-transfer flavoprotein]CATALYTIC ACTIVITY hexanoyl-CoA + oxidized [electron-transfer flavoprotein] + H(+) = (2E)-hexenoyl-CoA + reduced [electron-transfer flavoprotein]CATALYTIC ACTIVITY butanoyl-CoA + oxidized [electron-transfer flavoprotein] + H(+) = (2E)-butenoyl-CoA + reduced [electron-transfer flavoprotein]COFACTOR Amino-acid degradation; L-leucine degradation; (S)-3-hydroxy-3-methylglutaryl-CoA from 3-isovaleryl-CoA: step 1/3.SUBUNIT Homotetramer.INTERACTION The disease is caused by variants affecting the gene represented in this entry.SIMILARITY Belongs to the acyl-CoA dehydrogenase family.CAUTION It is uncertain whether Met-1 or Met-4 is the initiator.SEQUENCE CAUTION Truncated N-terminus.SEQUENCE CAUTION Truncated N-terminus.SEQUENCE CAUTION Truncated N-terminus.SEQUENCE CAUTION Truncated N-terminus.
databaseName UniProt
dbId 57639
description
  • recommendedName: fullName evidence="14"Isovaleryl-CoA dehydrogenase, mitochondrial shortName: IVD ecNumber evidence="10"1.3.8.4 alternativeName: Butyryl-CoA dehydrogenase ecNumber evidence="10"1.3.8.1
displayName UniProt:P26440 IVD
geneName
  • IVD
identifier P26440
isSequenceChanged false
keyword
  • 3D-structure
  • Acetylation
  • Alternative splicing
  • Direct protein sequencing
  • Disease variant
  • FAD
  • Fatty acid metabolism
  • Flavoprotein
  • Lipid metabolism
  • Mitochondrion
  • Oxidoreductase
  • Proteomics identification
  • Reference proteome
  • Transit peptide
moleculeType Protein
name
  • IVD
otherIdentifier
  • 11720642_a_at
  • 11720643_a_at
  • 16799538
  • 203682_PM_s_at
  • 203682_s_at
  • 216958_PM_s_at
  • 216958_s_at
  • 225311_PM_at
  • 225311_at
  • 3589906
  • 3589907
  • 3589908
  • 3589909
  • 3589910
  • 3589911
  • 3589912
  • 3589915
  • 3589916
  • 3589917
  • 3589918
  • 3589919
  • 3589920
  • 3589921
  • 3589922
  • 3589923
  • 3589924
  • 3589925
  • 3589926
  • 3589927
  • 3589928
  • 3589930
  • 3589931
  • 3589932
  • 3589933
  • 3589934
  • 3589935
  • 3589936
  • 3589937
  • 3589938
  • 3589944
  • 3712
  • 37342_s_at
  • 63548_at
  • 78746_at
  • 7982723
  • 81819_at
  • A_21_P0008622
  • A_23_P129313
  • A_23_P129322
  • A_33_P3269718
  • GE58276
  • GE86159
  • GE86160
  • GO:0003995
  • GO:0005515
  • GO:0005654
  • GO:0005739
  • GO:0005759
  • GO:0006520
  • GO:0006552
  • GO:0006629
  • GO:0006631
  • GO:0008470
  • GO:0009083
  • GO:0016491
  • GO:0016627
  • GO:0016937
  • GO:0019752
  • GO:0031966
  • GO:0033539
  • GO:0042802
  • GO:0050660
  • HMNXSV003014601
  • HMNXSV003025668
  • HMNXSV003033658
  • HMNXSV003042078
  • Hs.279789.5.A1_3p_at
  • Hs.279789.5.A1_3p_x_at
  • Hs.77510.2.S1_3p_a_at
  • ILMN_1724207
  • M34192_at
  • PH_hs_0024828
  • TC15000284.hg
  • TC15002158.hg
  • g6031167_3p_a_at
physicalEntity
schemaClass ReferenceGeneProduct
secondaryIdentifier
  • IVD_HUMAN
  • B2RCV5
  • B3KVI7
  • J3KR54
  • Q53XZ9
  • Q96AF6
sequenceLength 426
stId uniprot:P26440

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