Taking advantage of this high incidence, here we show that 1) DCBLD2 deficiency promotes the development of CAVD and aortic stenosis, which is more severe in animals with BAV, 2) the difference between Dcbld2−/− bicuspid and tricuspid valves is especially striking with regard to valvular calcification, which like in humans, affects the leaflets in these animals, and 3) BMP signaling is enhanced in BAV, despite a similarly up-regulated Bmp2 expression in Dcbld2−/− TAV and BAV. The gene discussed is BMP2; the disease is aortic stenosis.