
NSDHL Antibody (B-5) is a mouse monoclonal IgG2b antibody that detects NSDHL in human samples through various applications including western blotting (WB), immunoprecipitation (IP), immunofluorescence (IF), immunohistochemistry with paraffin-embedded sections (IHCP), and enzyme-linked immunosorbent assay (ELISA). NSDHL, or NAD(P) dependent steroid dehydrogenase-like, is a crucial 373 amino acid single-pass membrane protein predominantly expressed in the brain, heart, liver, lung, kidney, skin, and placenta. NSDHL is a member of the 3-beta-hydroxysteroid dehydrogenase family and is localized in the endoplasmic reticulum, where NSDHL plays a vital role in cholesterol biosynthesis. Specifically, NSDHL is involved in the demethylation of sterol precursors during one of the later stages of cholesterol synthesis, a process essential for maintaining cellular membrane integrity and fluidity, as well as for the production of steroid hormones. Deficiencies in the NSDHL gene can lead to congenital hemidysplasia with ichthyosiform erythroderma and limb defects (CHILD), an X-linked dominant disorder that severely impacts lipid metabolism and often results in male lethality. The clinical manifestations of CHILD include congenital hemidysplasia, ichthyosiform erythroderma, and hypoplasia of limbs and other skeletal structures, underscoring the importance of NSDHL in human health and development.
Ordering Information
| Product Name | Catalog # | UNIT | Price | Qty | FAVORITES | |
NSDHL Antibody (B-5) | sc-390994 | 200 µg/ml | $322.00 | |||
NSDHL Antibody (B-5): m-IgGκ BP-HRP Bundle | sc-552166 | 200 µg Ab; 40 µg BP | $361.00 |