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The process of transferring lipoic acid to proteins is a two-step process.
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LIPT1 protein is required for lipoylation and activation of 2-ketoacid dehydrogenases in humans.
Data report a putative case of impaired free or H protein (show MYBPH Proteins)-derived lipoic acid attachment due to LIPT1 mutations as a cause of PDH (show PDP Proteins) and alpha-KGDH deficiencies.
Results describe the crystal structure of bovine lipoyltransferase in complex with lipoyl-AMP (show TMPRSS5 Proteins).
The process of transferring lipoic acid to proteins is a two-step process. The first step is the activation of lipoic acid by lipoate-activating enzyme to form lipoyl-AMP. For the second step, the protein encoded by this gene transfers the lipoyl moiety to apoproteins. Alternative splicing results in multiple transcript variants. A related pseudogene has been identified on chromosome 13. Read-through transcription also exists between this gene and the neighboring downstream mitochondrial ribosomal protein L30 (MRPL30) gene.
, lipoyltransferase 1, mitochondrial-like
, lipoyltransferase 1, mitochondrial
, lipoate biosynthesis protein
, lipoyl ligase
, lipoate-protein ligase