Bi-allelic loss-of-function variants in LPL or in other genes promoting LPL activity in the capillary lumen (e.g., APOC2, APOA5, LMF1, GPIHBP1) cause severe hypertriglyceridemia with a predisposition to acute pancreatitis (6, 7). The gene discussed is LPL; the disease is hypertriglyceridemia.