产品描述:
AY 9944 prevents cholesterol biosynthesis by inhibiting the 7-dehydro cholesterol Δ7-reductase (DHCR7) enzyme (IC50 = 13 nM), which interferes with the conversion of 7-dehydro cholesterol (Item No. 14612) to cholesterol.{27278,27282} Furthermore, by upregulating the expression of DHCR7, AY 9944 can block Hedgehog signaling at the level of Smoothened or by loss of Suppressor of Fused.{27279} AY 9944 inhibition of DHCR7 has been used to recapitulate phenotypes of Smith-Lemli-Opitz syndrome, a disorder brought about by mutations in the DHCR7 gene, in animal models.{27282,27280} However, AY 9944 is highly teratogenic, producing congenital defects in offspring when fed to pregnant animals.{27281}