The molecular basis of premature atherosclerosis in the absence of high LDL-c levels is largely unknown, but recently rare and putative causal variants in Myocyte enhancer factor–2 (MEF2A) and Low-density lipoprotein receptor-related protein 6 (LRP6) have been identified in pedigrees with Mendelian forms of atherosclerosis where FH as a causal factor was ruled out [5], [10], [11]. Here, MEF2A is linked to atherosclerosis.