7-DHC/DHCR7 Recombinant Rabbit mAb (S-3625-32)

7-Dehydrocholesterol (7-DHC) is a crucial sterol intermediate in the cholesterol biosynthesis pathway that serves as the direct precursor to cholesterol and the provitamin for vitamin D3 synthesis upon exposure to UVB radiation, while DHCR7 (7-dehydrocholesterol reductase) is the essential membrane-bound enzyme located in the endoplasmic reticulum that catalyzes the final step of this pathway by reducing the C7-C8 double bond of 7-DHC to produce cholesterol; together, they form a tightly regulated metabolic axis where genetic defects or inhibition of DHCR7 lead to the pathological accumulation of 7-DHC and its derivatives, causing severe developmental disorders such as Smith-Lemli-Opitz syndrome (SLOS) characterized by intellectual disability and congenital malformations, whereas under normal physiological conditions, the precise balance maintained by DHCR7 ensures adequate cellular cholesterol levels for membrane integrity and steroid hormone production while simultaneously controlling the pool of 7-DHC available for cutaneous photconversion to vitamin D3.