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Details on Person UniProt:Q9Y234 LIPT1
| Class:Id | ReferenceGeneProduct:58581 |
|---|---|
| _chainChangeLog | transit peptide:1-25 added on Sat February 7 2015;chain:26-373 added on Sat February 7 2015 |
| _displayName | UniProt:Q9Y234 LIPT1 |
| _timestamp | 2026-02-20 22:45:24 |
| chain | transit peptide:1-25 chain:26-373 |
| checksum | 51E72E1E4F943C96 |
| comment | FUNCTION 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. |
| description | recommendedName: 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.- |
| geneName | LIPT1 |
| identifier | Q9Y234 |
| isSequenceChanged | FALSE |
| keyword | Acyltransferase 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 |
| name | LIPT1 |
| referenceDatabase | [ReferenceDatabase:2] UniProt |
| referenceGene | [ReferenceDNASequence:8998973] ENSEMBL:ENSG00000144182 LIPT1 [Homo sapiens] |
| secondaryIdentifier | LIPT_HUMAN Q4ZFZ1 |
| sequenceLength | 373 |
| species | [Species:48887] Homo sapiens |
| (referenceEntity) | [EntityWithAccessionedSequence:6792623] LIPT1 [mitochondrial matrix] [Homo sapiens] |
| [Change default viewing format] | |
No pathways have been reviewed or authored by UniProt:Q9Y234 LIPT1 (58581)
