LIPT2

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Description

The LIPT2 (lipoyl(octanoyl) transferase 2) is a protein-coding gene located on chromosome 11.

LIPT2 catalyzes the transfer of octanoic acid, produced within the cell, from octanoyl-acyl-carrier-protein (octanoyl-ACP) to the lipoyl domains of lipoate-dependent enzymes. These enzymes include the protein H of the glycine cleavage system (GCSH). While lipoyl-ACP can also be used as a substrate, octanoyl-ACP is considered the primary substrate in the body.

LIPT2 is also known as -.

Associated Diseases

• Encephalopathy, neonatal severe, with lactic acidosis and brain abnormalities