Angiopoietin-5 (ANG-5) is involved in lipid metabolism and plays a role in angiogenesis. Defects in ANG-5 are the cause of hypobetalipoproteinemia familial type 2 (FHBL2), also called combined hypobetalipoproteinemia familial. FHBL2 is a disorder of lipid metabolism characterized by less than 5th percentile age- and sex-specific levels of low density lipoproteins, and dietary fat malabsorption. Affected individuals present with combined hypolipidemia, consisting of extremely low plasma levels of LDL cholesterol, HDL cholesterol, and triglycerides.