LIPT2 (lipoyl(octanoyl) transferase 2) is a mitochondrial enzyme that catalyzes the transfer of octanoic acid from octanoyl-acyl-carrier-protein (octanoyl-ACP) to lipoyl domains of lipoate-dependent enzymes, initiating lipoic acid assembly on protein cofactors 1. This process is essential for lipoylating the H protein of the glycine cleavage system and the E2 subunits of alpha-ketoglutarate dehydrogenase, which are critical for mitochondrial energy metabolism 23. LIPT2 functions as part of a three-enzyme pathway with LIAS and LIPT1 that synthesizes and attaches lipoic acid cofactors to five redox-active enzymatic complexes 3. Mutations in LIPT2 cause severe neonatal encephalopathy with lactic acidosis and brain abnormalities, reflecting the vital role of lipoic acid in cellular respiration 4. In Huntington's disease, LIPT2 degradation via the E3 ubiquitin ligase NEDD4L impairs mitochondrial function and neuronal survival; restoring LIPT2 levels or supplementing lipoic acid mitigates disease phenotypes 2. Emerging evidence indicates LIPT2 dysregulation may influence cancer progression and prognosis across multiple tumor types 56. LIPT2 represents a targetable node in lipoic acid metabolism for treating mitochondrial and neurodegenerative diseases 7.