Genetic factors account for 50% of individual variation in plasma triglyceride (TG) levels (Goldberg et al., 2011; Miller et al., 2011; Namboodiri et al., 1985), whereas severe hypertriglyceridemia (TG>885 mg/dl) is usually caused by single mutations in the gene encoding lipoprotein lipase (LPL), or less commonly, in genes affecting proteins involved in LPL activity, such as apolipoprotein C-II (APOC2), lipase maturation factor 1 (LMF1), apolipoprotein A-V (APOA5) and GPI HDL binding protein1 (GPIHBP1) (Surendran et al., 2012). The gene discussed is APOC2; the disease is hypertriglyceridemia.