LIPT1


Description

The LIPT1 (lipoyltransferase 1) is a protein-coding gene located on chromosome 2.

LIPT1, also known as Lipoate biosynthesis protein, Lipoate-protein ligase, Lipoyl ligase, and Lipoyltransferase 1, is a lipoyl amidotransferase that catalyzes the transfer of lipoyl groups from lipoyl-protein H (lipoyl-GCSH) of the glycine cleavage system to E2 subunits of the pyruvate dehydrogenase complex (PDCE2). It is unable to transfer octanoyl groups from octanoyl-GCSH to PDCE2. In laboratory settings, LIPT1 can also transfer the lipoyl group from lipoyl-AMP to specific lysine residues in the lipoyl domains of lipoate-dependent enzymes, but this reaction may not be significant in the body.

LIPT1 is also known as LIPT1D.

Associated Diseases


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