Lipoyl(octanoyl) transferase 2 (LIPT2)

Lipoyl(octanoyl) transferase 2 (LIPT2) is a mitochondrial enzyme crucial for the post-translational lipoylation of lipoate-dependent proteins, thereby supporting key steps in energy metabolism. LIPT2 catalyzes the transfer of octanoic acid, derived from octanoyl-acyl-carrier-protein (octanoyl-ACP), to the lipoyl domains of target proteins, representing the first dedicated step in lipoic acid biosynthesis in humans. Correct mitochondrial targeting, determined by an N-terminal mitochondrial presequence, is essential for its activity; mis-targeting disrupts mitochondrial function and can lead to activation of apoptotic pathways, mitochondrial membrane collapse, and caspase-3 activation. Genetic defects in LIPT2 cause severe, early-onset metabolic diseases, including neonatal severe encephalopathies and cortical brain malformations. There are currently no approved drugs or clinical inhibitors of LIPT2, but mutations in this gene serve as important diagnostic biomarkers for specific mitochondrial disorders.

Other names

Lipoyl transferase 2Octanoyl-[acyl-carrier-protein]:protein N-octanoyltransferase LIPT2, mitochondrialLipoate-protein ligase BLipoyl/octanoyl transferaseLipoyltransferase 2Octanoyl-[acyl-carrier-protein]-protein N-octanoyltransferaselipoyl(octanoyl) transferase 2 (putative)putative lipoyltransferase 2, mitochondrialputative octanoyltransferase, mitochondrial

Not applicable; there are no approved drugs or clinical-stage agents directly targeting LIPT2