Rabbit anti-Human, Mouse EBP Polyclonal Antibody | anti-EBP antibody

EBP, NT (Emopamil-binding Protein, Delta(8)-Delta(7) Sterol Isomerase, D8-D7 Sterol Isomerase, Cholestenol Delta-isomerase, 3-beta-hydroxysteroid-Delta(8),Delta(7)-isomerase) (Biotin)

Cat. Num. AAA6473455

• Gene Names: EBP; CPX; CHO2; CPXD; CDPX2
• Reactivity: Human, Mouse
• Applications: Western Blot, ELISA
• Purity: Purified by Immunoaffinity chromatography.
• Synonyms: EBP; Polyclonal Antibody; NT (Emopamil-binding Protein; Delta(8)-Delta(7) Sterol Isomerase; D8-D7 Sterol Isomerase; Cholestenol Delta-isomerase; 3-beta-hydroxysteroid-Delta(8); Delta(7)-isomerase) (Biotin); anti-EBP antibody

• Host: Rabbit
• Reactivity: Human, Mouse
• Clonality: Polyclonal
• Isotype: IgG
• Specificity: Recognizes human EBP. Species Crossreactivity: mouse.
• Purity/Purification: Purified by Immunoaffinity chromatography.
• Form/Format: Supplied as a liquid in PBS, pH 7.2. No preservative added. Labeled with Biotin.

• Applicable Applications for anti-EBP antibody: Western Blot (WB), ELISA (EIA)
• Application Notes: WB: 1:100-1:500; ELISA: 1:1,000; Applications are based on unconjugated antibody.
• Immunogen: Synthetic peptide selected from the N-terminal region of human EBP (KLH).
• Conjugate: Biotin
• Note: Preservative Free
• Preparation and Storage: Store product at 4 degree C if to be used immediately within two weeks. For long-term storage, aliquot to avoid repeated freezing and thawing and store at -20 degree C. Aliquots are stable at -20 degree C for 12 months after receipt. Dilute required amount only prior to immediate use. Further dilutions can be made in assay buffer. For maximum recovery of product, centrifuge the original vial after thawing and prior to removing the cap.

Western Blot (WB)

(Western blot analysis of EBP Antibody (N-term) in mouse kidney tissue lysates (35ug/lane). EBP (arrow) was detected using the purified Pab.)

Immunohistochemistry (IHC)

(Formalin-fixed and paraffin-embedded human hepatocarcinoma reacted with EBP Antibody (N-term), which was peroxidase-conjugated to the secondary antibody, followed by DAB staining. This data demonstrates the use of this antibody for immunohistochemistry; clinical relevance has not been evaluated.)

Flow Cytometry (FC/FACS)

(EBP Antibody (N-term) flow cytometric analysis of Hela cells (right histogram) compared to a negative control cell (left histogram).FITC-conjugated goat-anti-rabbit secondary antibodies were used for the analysis.)

Product Categories/Family for anti-EBP antibody

Antibodies; Enzymes; Isomerase

References

1. Rakheja, D., et.al., Pediatr. Dev. Pathol. 10(2), 142-148 (2007).

NCBI and Uniprot Product Information

• NCBI GI #: 5729810
• NCBI GeneID: 10682
• NCBI Accession #: NP_006570.1
• NCBI GenBank Nucleotide #: NM_006579.2
• UniProt Accession #: Q15125; Q6FGL3; Q6IBI9
• Molecular Weight: 26,353 Da
• NCBI Official Full Name: 3-beta-hydroxysteroid-Delta(8),Delta(7)-isomerase
• NCBI Official Synonym Full Names: emopamil binding protein (sterol isomerase)
• NCBI Official Symbol: EBP
• NCBI Official Synonym Symbols: CPX; CHO2; CPXD; CDPX2
• NCBI Protein Information: 3-beta-hydroxysteroid-Delta(8),Delta(7)-isomerase; 3-beta-hydroxysteroid-Delta(8),Delta(7)-isomerase; sterol 8-isomerase; D8-D7 sterol isomerase; cholestenol Delta-isomerase; delta(8)-Delta(7) sterol isomerase; emopamil-binding protein (sterol isomerase)
• UniProt Protein Name: 3-beta-hydroxysteroid-Delta(8),Delta(7)-isomerase
• Protein Family: Ejaculatory bulb-specific protein
• UniProt Gene Name: EBP
• UniProt Synonym Gene Names: D8-D7 sterol isomerase
• UniProt Entry Name: EBP_HUMAN

NCBI Description

The protein encoded by this gene is an integral membrane protein of the endoplasmic reticulum. It is a high affinity binding protein for the antiischemic phenylalkylamine Ca2+ antagonist [3H]emopamil and the photoaffinity label [3H]azidopamil. It is similar to sigma receptors and may be a member of a superfamily of high affinity drug-binding proteins in the endoplasmic reticulum of different tissues. This protein shares structural features with bacterial and eukaryontic drug transporting proteins. It has four putative transmembrane segments and contains two conserved glutamate residues which may be involved in the transport of cationic amphiphilics. Another prominent feature of this protein is its high content of aromatic amino acid residues (>23%) in its transmembrane segments. These aromatic amino acid residues have been suggested to be involved in the drug transport by the P-glycoprotein. Mutations in this gene cause Chondrodysplasia punctata 2 (CDPX2; also known as Conradi-Hunermann syndrome). [provided by RefSeq, Jul 2008]

Uniprot Description

EBP: Catalyzes the conversion of Delta(8)-sterols to their corresponding Delta(7)-isomers. Defects in EBP are the cause of chondrodysplasia punctata X-linked dominant type 2 (CDPX2); also known as Conradi-Hunermann-Happle syndrome. CDP is a clinically and genetically heterogeneous disorder characterized by punctiform calcification of the bones. The key clinical features of CDPX2 are chondrodysplasia punctata, linear ichthyosis, cataracts and short stature. CDPX2 is a rare disorder of defective cholesterol biosynthesis, biochemically characterized by an increased amount of 8-dehydrocholesterol and cholest-8(9)-en-3-beta-ol in the plasma and tissues. Belongs to the EBP family.

Protein type: Membrane protein, multi-pass; Isomerase; Endoplasmic reticulum; Lipid Metabolism - steroid biosynthesis; EC 5.3.3.5; Membrane protein, integral

Chromosomal Location of Human Ortholog: Xp11.23-p11.22

Cellular Component: endoplasmic reticulum membrane; endoplasmic reticulum; integral to plasma membrane

Molecular Function: transmembrane receptor activity; steroid delta-isomerase activity; cholestenol delta-isomerase activity; drug transporter activity; C-8 sterol isomerase activity

Biological Process: cholesterol metabolic process; multidrug transport; hemopoiesis; signal transduction; skeletal development; cholesterol biosynthetic process

Disease: Chondrodysplasia Punctata 2, X-linked Dominant