Homozygous FH (hoFH) patients display severe hypercholesterolemia with accelerated atherosclerotic CHD in childhood and, without treatment, death usually occurs at the end of the second decade of life.2 LDLR binds and removes LDL-cholesterol from the bloodstream via receptor-mediated endocytosis. This evidence concerns the gene LDLR and familial hypercholesterolemia.