Owing to mutations in both alleles of the LDLR locus, homozygous LDLR-associated FH patients present with markedly elevated total serum cholesterol (>500 mg/dL, 13 mmol/L) and LDL-cholesterol levels (LDL-C, >450 mg/dL, 11.7 mmol/L). This evidence concerns the gene LDLR and familial hyperaldosteronism.