LMF1 (lipase maturation factor 1) is an endoplasmic reticulum chaperone protein essential for the posttranslational maturation of lipoprotein lipase (LPL) and hepatic lipase 1. Each LMF1 molecule facilitates the maturation of approximately 50 or more LPL molecules through processes involving glycosylation, folding, and subunit assembly 1. LMF1 variation directly modulates LPL activity in vivo; transgenic overexpression increases LPL activity in adipose and muscle tissues 1. Loss-of-function mutations in LMF1 cause familial chylomicronemia syndrome (FCS), characterized by severely elevated plasma triglycerides (>10 mmol/L) and acute pancreatitis risk 2. LMF1 mutations account for approximately 1% of FCS cases 3, though compound heterozygous and homozygous variants have been documented 34. Heterozygous LMF1 variants contribute to multifactorial chylomicronemia, a more common form of genetic hypertriglyceridemia 5. Specific variants (p.Trp464Ter, p.Gly172Arg, p.Arg354Trp) reduce or abolish LPL activity in functional studies 4. Clinical detection of LMF1-related hypertriglyceridemia involves genetic testing and assessment of postheparin LPL activity, with management including very-low-fat diets and emerging biotechnological therapies 6.