Anti-NSDHL / Sterol-4-alpha-carboxylate 3-dehydrogenase

Adding 0.2 ml of distilled water will yield a concentration of 500 ug/ml. NSDHL antibody detects Sterol-4-alpha-carboxylate 3-dehydrogenase, decarboxylating, a membrane-associated enzyme involved in cholesterol biosynthesis. The UniProt recommended name is Sterol-4-alpha-carboxylate 3-dehydrogenase, decarboxylating (NSDHL). This enzyme catalyzes oxidative decarboxylation of sterol intermediates during the post-squalene steps of cholesterol production, playing a crucial role in lipid homeostasis and membrane integrity.Functionally, NSDHL antibody identifies a 373-amino-acid enzyme localized to the endoplasmic reticulum membrane. NSDHL acts sequentially with other enzymes such as HSD17B7 and EBP to remove carboxyl groups from C4-methyl sterols, facilitating the conversion of lanosterol to cholesterol. Its catalytic activity contributes to maintaining sterol balance essential for cell signaling and membrane function.The NSDHL gene is located on chromosome Xq28 and is highly expressed in liver, skin, and brain tissues. Its activity supports embryonic development, epidermal differentiation, and lipid raft formation in cell membranes. NSDHL is evolutionarily conserved and tightly regulated to ensure steady cholesterol output.Pathologically, mutations in NSDHL cause CHILD syndrome (Congenital Hemidysplasia with Ichthyosiform erythroderma and Limb Defects), a rare X-linked disorder involving defective cholesterol metabolism and asymmetric skin and limb malformations. NSDHL dysfunction also disrupts sterol intermediates, leading to toxic accumulation of precursors. Research using NSDHL antibody supports studies in lipid metabolism, cholesterol biosynthesis, and genetic disorders of sterol processing.NSDHL antibody is validated for western blotting, immunohistochemistry, and immunofluorescence to detect sterol biosynthetic enzymes. NSJ Bioreagents provides NSDHL antibody reagents optimized for lipid biology, metabolism, and enzymology research.Structurally, Sterol-4-alpha-carboxylate 3-dehydrogenase, decarboxylating contains a short-chain dehydrogenase/reductase catalytic domain and transmembrane segments that anchor it to the endoplasmic reticulum. This antibody enables study of NSDHL's role in sterol conversion and metabolic regulation.