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Details on Person UniProt:Q9Y234 LIPT1

Class:IdReferenceGeneProduct:58581
_chainChangeLogtransit peptide:1-25 added on Sat February 7 2015;chain:26-373 added on Sat February 7 2015
_displayNameUniProt:Q9Y234 LIPT1
_timestamp2026-02-20 22:45:24
chaintransit peptide:1-25
chain:26-373
checksum51E72E1E4F943C96
commentFUNCTION Lipoyl amidotransferase that catalyzes the transfer of lipoyl moieties from lipoyl-protein H of the glycine cleavage system (lipoyl-GCSH) to E2 subunits of the pyruvate dehydrogenase complex (PDCE2) (PubMed:29987032). Unable to catalyze the transfer of octanoyl from octanoyl-GCSH to PDCE2 (PubMed:29987032). In vitro, it is also able to catalyze the transfer of the lipoyl group from lipoyl-AMP to the specific lysine residue of lipoyl domains of lipoate-dependent enzymes but this reaction may not be physiologically relevant (Probable).CATALYTIC ACTIVITY N(6)-[(R)-lipoyl]-L-lysyl-[glycine-cleavage complex H protein] + L-lysyl-[lipoyl-carrier protein] = L-lysyl-[glycine-cleavage complex H protein] + N(6)-[(R)-lipoyl]-L-lysyl-[lipoyl-carrier protein]CATALYTIC ACTIVITY (R)-lipoyl-5'-AMP + L-lysyl-[lipoyl-carrier protein] = N(6)-[(R)-lipoyl]-L-lysyl-[lipoyl-carrier protein] + AMP + 2 H(+)PATHWAY Protein modification; protein lipoylation via exogenous pathway; protein N(6)-(lipoyl)lysine from lipoate: step 2/2.SUBCELLULAR LOCATION Highly expressed in skeletal muscle and heart, moderately in kidney and pancreas, and detected at lower levels in liver, brain, placenta and lung.DISEASE The disease is caused by variants affecting the gene represented in this entry.SIMILARITY Belongs to the LplA family.
descriptionrecommendedName: fullName evidence="10"Lipoyl amidotransferase LIPT1, mitochondrial ecNumber evidence="8"2.3.1.200 alternativeName: Lipoate biosynthesis protein alternativeName: Lipoate-protein ligase alternativeName: Lipoyl ligase alternativeName: Lipoyltransferase 1 ecNumber evidence="2"2.3.1.-
geneNameLIPT1
identifierQ9Y234
isSequenceChangedFALSE
keywordAcyltransferase
Disease variant
Mitochondrion
Proteomics identification
Reference proteome
Transferase
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:9926675] Weiser, Joel, 2024-11-03
[InstanceEdit:9939033] Weiser, Joel, 2025-02-21
[InstanceEdit:9983091] Weiser, Joel, 2026-02-20
nameLIPT1
referenceDatabase[ReferenceDatabase:2] UniProt
referenceGene[ReferenceDNASequence:8998973] ENSEMBL:ENSG00000144182 LIPT1 [Homo sapiens]
secondaryIdentifierLIPT_HUMAN
Q4ZFZ1
sequenceLength373
species[Species:48887] Homo sapiens
(referenceEntity)[EntityWithAccessionedSequence:6792623] LIPT1 [mitochondrial matrix] [Homo sapiens]
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