In most cases, FH is caused by a mutation in the LDL receptor gene (LDL-R gene), resulting in absent or dysfunctional receptors on the surface of hepatocytes and a reduced liver capacity to remove atherogenic cholesterol-rich low-density lipoproteins (LDLs) from the circulation, with consequent accumulation of LDL cholesterol (LDL-C). The gene discussed is LDLR; the disease is familial hyperaldosteronism.