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Details on Person UniProt:Q23571 dbt-1

Class:IdReferenceGeneProduct:3445784
_chainChangeLogtransit peptide:1- added on Fri February 6 2015;chain:-448 added on Fri February 6 2015
_displayNameUniProt:Q23571 dbt-1
_timestamp2026-02-20 22:46:49
chaintransit peptide:1-
chain:-448
checksumE3C0FC5B2C5946AA
commentFUNCTION The branched-chain alpha-keto dehydrogenase complex catalyzes the overall conversion of alpha-keto acids to acyl-CoA and CO(2) (By similarity). It contains multiple copies of three enzymatic components: branched-chain alpha-keto acid decarboxylase (E1), lipoamide acyltransferase (E2) and lipoamide dehydrogenase (E3) (By similarity). Within this complex, the catalytic function of this enzyme is to accept, and to transfer to coenzyme A, acyl groups that are generated by the branched-chain alpha-keto acid decarboxylase component (By similarity). Required for the catabolism of branched-chain amino acids and the subsequent synthesis of monomethyl branched-chain fatty acids, which are important for regulating postembryonic growth (PubMed:26683372).CATALYTIC ACTIVITY N(6)-[(R)-dihydrolipoyl]-L-lysyl-[protein] + 2-methylpropanoyl-CoA = N(6)-[(R)-S(8)-2-methylpropanoyldihydrolipoyl]-L-lysyl-[protein] + CoACATALYTIC ACTIVITY N(6)-[(R)-dihydrolipoyl]-L-lysyl-[protein] + 3-methylbutanoyl-CoA = N(6)-[(R)-S(8)-3-methylbutanoyldihydrolipoyl]-L-lysyl-[protein] + CoACATALYTIC ACTIVITY (2S)-2-methylbutanoyl-CoA + N(6)-[(R)-dihydrolipoyl]-L-lysyl-[protein] = N(6)-{(R)-S(8)-[(2S)-2-methylbutanoyl]dihydrolipoyl}-L-lysyl-[protein] + CoACOFACTOR Binds 1 lipoyl cofactor covalently.SUBCELLULAR LOCATION Localizes to puncta within the cytosol which is consistent with its expected location in mitochondria.TISSUE SPECIFICITY Ubiquitously expressed.DISRUPTION PHENOTYPE RNAi-mediated knockdown results in reduced monomethyl branched-chain fatty acid generation and also results in larval arrest in the subsequent generation.SIMILARITY Belongs to the 2-oxoacid dehydrogenase family.
created[InstanceEdit:3445779] Weiser, JD
descriptionrecommendedName: fullName evidence="8"Lipoamide acyltransferase component of branched-chain alpha-keto acid dehydrogenase complex, mitochondrial ecNumber evidence="1"2.3.1.168 alternativeName: Branched-chain alpha-keto acid dehydrogenase complex component E2 alternativeName: fullName evidence="9"Dihydrolipoamide branched-chain transacylase E2
geneNamedbt-1
ZK669.4
identifierQ23571
isSequenceChangedFALSE
keywordAcyltransferase
Cell projection
Cytoplasm
Fatty acid biosynthesis
Fatty acid metabolism
Lipid biosynthesis
Lipid metabolism
Lipoyl
Mitochondrion
Reference proteome
Transferase
Transit peptide
modified[InstanceEdit:4341137] Weiser, JD
[InstanceEdit:5433710] Weiser, JD
[InstanceEdit:5618415] Weiser, JD
[InstanceEdit:5634237] Weiser, JD
[InstanceEdit:5673015] Weiser, JD
[InstanceEdit:8964659] Weiser, JD
[InstanceEdit:8987656] Weiser, JD
[InstanceEdit:9037114] Weiser, JD
[InstanceEdit:9637257] Weiser, JD
[InstanceEdit:9676415] Weiser, JD
[InstanceEdit:9750299] Weiser, JD
[InstanceEdit:9852000] Weiser, Joel, 2023-11-03
[InstanceEdit:9862192] Weiser, Joel, 2024-02-26
[InstanceEdit:9939033] Weiser, Joel, 2025-02-21
[InstanceEdit:9983091] Weiser, Joel, 2026-02-20
namedbt-1
referenceDatabase[ReferenceDatabase:2] UniProt
secondaryIdentifierODB2_CAEEL
sequenceLength448
species[Species:68320] Caenorhabditis elegans
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