AGPAT2 encodes 1-acylglycerol-3-phosphate O-acyltransferase 2, a critical enzyme catalyzing the conversion of lysophosphatidic acid (LPA) to phosphatidic acid (PA) at the sn-2 position of the glycerol backbone 1. This reaction is essential for triglyceride and phospholipid synthesis via the glycerol-3-phosphate pathway 2. AGPAT2 is uniquely required for adipocyte development and maturation among AGPAT isoforms 1. Beyond canonical lipid synthesis, AGPAT2-generated PA drives endoplasmic reticulum tubulation through direct interaction with the dynamin-related GTPase DRP1, independent of DRP1's canonical mitochondrial division function 3. Pathogenic AGPAT2 variants cause Congenital Generalized Lipodystrophy type 1 (CGL1), characterized by absent fatty tissue from birth 1. CGL1 patients develop severe metabolic complications including hypoleptinemia, hypoadiponectinemia, hyperglycemia, and hypertriglyceridemia 1. AGPAT2 deficiency is also associated with neonatal diabetes mellitus in some patients 4. Animal models show AGPAT2 deficiency exacerbates hyperlipidemia, hepatic steatosis, and atherosclerosis progression 5. Loss-of-function mutations typically reduce AGPAT2 protein stability rather than affecting mRNA levels, suggesting post-translational degradation mechanisms 6.