Abcam, ab246864, Anti-emopamil binding protein antibody

Size: 100µL
Rabbit Polyclonal emopamil binding protein antibody. Suitable for IHC-P, ICC/IF and reacts with Human samples. Cited in 1 publication. Immunogen corresponding to Recombinant Fragment Protein within Human EBP aa 50-150.
Key facts
Host species:Rabbit,
Clonality:Polyclonal,
Isotype:IgG,
Carrier free:No,
Reacts with:Human,
Applications:ICC/IF, IHC-PSee reactivity dataSee the reactivity data table below for information on validated species and application combinations.,
Immunogen:Recombinant Fragment Protein within Human EBP aa 50-150. The exact immunogen used to generate this antibody is proprietary information.Q15125

Properties and Storage Information:
Form-Liquid, Purification technique-Affinity purification Immunogen, Storage buffer-pH: 7.2Preservative: 0.02% Sodium azideConstituents: PBS, 40% 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.
Emopamil binding protein also known as EBP is a membrane protein with a molecular mass of approximately 27 kDa. EBP functions as a high-affinity binding site for emopamil and is involved in the catalytic activity of the enzyme sterol isomerase. It is mainly expressed in various tissues including the liver brain and skin indicating its broad role in the body. This protein plays a significant role in the conversion of sterol intermediates important for cholesterol biosynthesis.
Biological function summary
The emopamil binding protein contributes to the sterol biosynthesis pathway by catalyzing the isomerization of delta8-sterols to delta7-sterols. It functions as a part of a larger enzyme complex being vital in producing cholesterol and other essential sterols. EBP's enzymatic role supports cellular membrane integrity and impacts several physiological processes such as hormone production and energy metabolism.
Pathways
The participation of emopamil binding protein in the cholesterol biosynthesis pathway stands out which is essential for maintaining cellular function and structure. It also plays a role in the synthesis of steroid hormones like estrogen and testosterone. EBP's function is aligned with enzymes such as lanosterol synthase and squalene epoxidase both of which are integral components in the sterol and isoprenoid biosynthetic pathways.
Mutations or dysfunctions in emopamil binding protein have strong links to diseases like Chondrodysplasia punctata 2 (Conradi–Hünermann syndrome) and X-linked dominant Conradi–Hünermann syndrome. This condition is marked by skeletal malformations and skin abnormalities. The connection of EBP to these disorders often involves its interaction with sterol regulatory element-binding proteins (SREBPs) which regulate the expression of genes involved in lipid biosynthesis and homeostasis.