LDLRAP1 and autosomal dominant disease: FH is an autosomal dominant disease, primarily due to mutations in four genes: i) LDL-R LOF mutations, accounting for >90% of patients; ii) ApoB100 LOF mutations (Innerarity et al., 1987); iii) PCSK9 gain-of-function (GOF) mutations (Abifadel et al., 2003); and iv) low-density lipoprotein receptor adaptor protein 1 (LDLRAP1) LOF mutations, the only ones leading to a recessive form (Tada et al., 2011).