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Details on Person UniProt:Q9UKU7 ACAD8

Class:Id	ReferenceGeneProduct:49486
_chainChangeLog	transit peptide:1-22 added on Fri February 6 2015;chain:23-415 added on Fri February 6 2015
_displayName	UniProt:Q9UKU7 ACAD8
_timestamp	2025-05-21 21:53:42
chain	transit peptide:1-22 chain:23-415
checksum	CAFFE91B74E2362D
comment	FUNCTION Isobutyryl-CoA dehydrogenase which catalyzes the conversion of 2-methylpropanoyl-CoA to (2E)-2-methylpropenoyl-CoA in the valine catabolic pathway (PubMed:11013134, PubMed:12359132, PubMed:16857760). To a lesser extent, also able to catalyze the oxidation of (2S)-2-methylbutanoyl-CoA (PubMed:11013134, PubMed:12359132).CATALYTIC ACTIVITY 2-methylpropanoyl-CoA + oxidized [electron-transfer flavoprotein] + H(+) = 2-methylpropenoyl-CoA + reduced [electron-transfer flavoprotein]CATALYTIC ACTIVITY (2S)-2-methylbutanoyl-CoA + oxidized [electron-transfer flavoprotein] + H(+) = (2E)-2-methylbut-2-enoyl-CoA + reduced [electron-transfer flavoprotein]CATALYTIC ACTIVITY propanoyl-CoA + oxidized [electron-transfer flavoprotein] + H(+) = acryloyl-CoA + reduced [electron-transfer flavoprotein]COFACTOR kcat is 2.0 sec(-1) for the oxidation of 2-methylpropanoyl-CoA (PubMed:12359132). kcat is 4.1 sec(-1) for the oxidation of (2S)-methylbutanoyl-CoA (PubMed:12359132). kcat is 0.83 sec(-1) for the oxidation of n-propionyl-CoA (PubMed:12359132).PATHWAY Amino-acid degradation; L-valine degradation.SUBUNIT Homotetramer, formed by a dimer of dimers (PubMed:11013134, PubMed:14752098). May be part of the large multiprotein complex ARC/DRIP (PubMed:10235267).INTERACTION Detected at comparable levels in heart, lung, brain, skeletal muscle, pancreas and placenta. Weakly expressed in liver and kidney.DISEASE The disease is caused by variants affecting the gene represented in this entry.SIMILARITY Belongs to the acyl-CoA dehydrogenase family.
description	recommendedName: Isobutyryl-CoA dehydrogenase, mitochondrial shortName evidence="11"IBDH ecNumber evidence="5 6 9"1.3.8.5 alternativeName: Activator-recruited cofactor 42 kDa component shortName: ARC42 alternativeName: fullName evidence="10"Acyl-CoA dehydrogenase family member 8 shortName evidence="10"ACAD-8
geneName	ACAD8 ARC42 IBD
identifier	Q9UKU7
isSequenceChanged	FALSE
keyword	3D-structure Acetylation Activator Alternative splicing Branched-chain amino acid catabolism Direct protein sequencing Disease variant FAD Flavoprotein Mitochondrion Oxidoreductase Proteomics identification Reference proteome Transcription Transcription regulation 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	ACAD8
referenceDatabase	[ReferenceDatabase:2] UniProt
referenceGene	[ReferenceDNASequence:8998135] ENSEMBL:ENSG00000151498 ACAD8 [Homo sapiens]
secondaryIdentifier	ACAD8_HUMAN B7Z5W4 Q6ZWP6 Q9BUS8
sequenceLength	415
species	[Species:48887] Homo sapiens
(isoformParent)	[ReferenceIsoform:8969062] UniProt:Q9UKU7-1 ACAD8 [Homo sapiens] [ReferenceIsoform:8969063] UniProt:Q9UKU7-2 ACAD8 [Homo sapiens] [ReferenceIsoform:8969064] UniProt:Q9UKU7-3 ACAD8 [Homo sapiens]
(referenceEntity)	[EntityWithAccessionedSequence:70855] ACAD8 [mitochondrial matrix] [Homo sapiens]
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No pathways have been reviewed or authored by UniProt:Q9UKU7 ACAD8 (49486)