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Objective: Severe hypertriglyceridemia is associated with an increase risk for acute pancreatitis.Genetic determinants of severe hypertriglyceridemia in Thai subjects are incompletely understood.APOA5 gene, encoding apo A-5, is a newly discovered gene implicated in triglyceride metabolism.In this study, we examined genetic variations in the APOA5 gene in Thai subjects with severe hypertriglyceridemia.Design and Methods: The coding region and exonintron junctions of the APOA5 gene were examined by sequencing in 50 Thai subjects with severe hypertriglyceridemia (triglyceride levels more than 10mmol/L [886mg/dL] on≥2 occasions) and 61 controls.Results: We identified 6 variants in the APOA5 gene as shown below.Conclusions: Both known and novel variants in the APOA5 gene were identified.Increased prevalence ofc.-3A>G and c.553G>T variants in the severe hypertriglyceridemic group may contribute to this phenotype in Thai subjects.