Abcam, ab237799, Anti-NSDHL antibody

Size: 100µL
Rabbit Polyclonal NSDHL antibody. Suitable for IHC-P, ICC/IF and reacts with Human samples. Cited in 1 publication. Immunogen corresponding to Recombinant Fragment Protein within Human NSDHL aa 150-250.
Key facts
Host species:Rabbit,
Clonality:Polyclonal,
Isotype:IgG,
Carrier free:No,
Reacts with:Human,
Applications:IHC-P, ICC/IFSee reactivity dataSee the reactivity data table below for information on validated species and application combinations.,
Immunogen:Recombinant Fragment Protein within Human NSDHL aa 150-250. The exact immunogen used to generate this antibody is proprietary information.Q15738

Properties and Storage Information:
Form-Liquid, Purification technique-Affinity purification Protein G, Purification notes-Purity greater than 95%., Storage buffer-pH: 7.4Preservative: 0.03% Proclin 300Constituents: PBS, 50% Glycerol (glycerin, glycerine), Shipped at conditions-Blue Ice, Appropriate short-term storage duration-1-2 weeks, Appropriate short-term storage conditions-+4°C, Appropriate long-term storage conditions--20°C, Aliquoting information-Upon delivery aliquot, Storage information-Avoid freeze / thaw cycle

Supplementary Information:
This supplementary information is collated from multiple sources and compiled automatically.
NSDHL also known as NAD(P)H steroid dehydrogenase-like is an enzyme linked to the cholesterol biosynthesis pathway. It has a molecular weight of approximately 41 kDa. This enzyme localizes to the endoplasmic reticulum membrane and the peroxisomes. NSDHL shows expression in various tissues with higher levels in the liver adrenal glands and the brain. This distribution highlights its essential role in metabolic processes across different tissue types.
Biological function summary
NSDHL facilitates the removal of one hydrogen from NAD(P)H and adds it to the steroid precursor lathosterol an important intermediate in cholesterol biosynthesis. Although not part of a larger complex NSDHL performs significant enzymatic steps necessary for converting sterol intermediates. Its activity directly influences sterol composition vital for cell membrane integrity signaling and hormone synthesis.
Pathways
The enzyme plays a critical role in the cholesterol biosynthesis pathway specifically affecting the conversion of lanosterol into cholesterol. NSDHL operates in tandem with other enzymes like DHCR7 influencing the downstream process of sterol maturation. The precise coordination of these enzymes ensures effective cholesterol production essential for maintaining cellular cholesterol levels and systemic lipid homeostasis.
Malfunctions in NSDHL can lead to disorders like CHILD syndrome (Congenital Hemidysplasia with Ichthyosiform erythroderma and Limb Defects) and CK syndrome (Conradi-Hünermann-Happle syndrome). These conditions link to cholesterol biosynthesis disruption impacting skin and skeletal development. NSDHL interacts with the EBP protein in these pathways suggesting a relationship between enzyme activity and phenotypic expression in these disorders. Understanding NSDHL's function and regulation is key to recognizing its involvement in metabolic syndromes and potential therapeutic targets.