AGK (acylglycerol kinase) is a lipid kinase that phosphorylates monoacylglycerols and diacylglycerols to produce lysophosphatidic acid (LPA) and phosphatidic acid (PA), respectively 1. It also phosphorylates ceramide and shows substrate preference for 1,2-dioleoylglycerol over regioisomers 1. Beyond its kinase function, AGK acts as a kinase-independent component of the TIM22 mitochondrial import complex, mediating insertion of multi-pass transmembrane proteins into the mitochondrial inner membrane 23. Within TIM22, AGK is specifically required for importing metabolite carriers like ANT1 and SLC25A24 2. AGK overexpression increases LPA formation and secretion, activating EGFR and downstream MAPK signaling to promote cell growth 1. AGK deficiency impairs mitochondrial complex I function and is associated with non-alcoholic steatohepatitis (NASH) progression through disrupted mitochondrial fatty acid metabolism 4. Clinically, AGK mutations cause Mitochondrial DNA Depletion Syndrome 10, characterized by impaired mtDNA synthesis and systemic organ dysfunction 5. AGK mutations also cause Sengers syndrome, presenting with congenital cataracts, cardiomyopathy, myopathy, and lactic acidosis 4. These disease associations underscore AGK's critical dual role in lipid metabolism and mitochondrial protein import.