Anti-HSD3B7 Antibody Picoband®

SKU: A10025-1

Size: 100 μg

Storage: At -20℃ for one year from date of receipt. After reconstitution, at 4℃ for one month. It can also be aliquotted and stored frozen at -20℃ for six months. Avoid repeated freezing and thawing.

Form: Lyophilized

Reactivity: Human

Applications: WB,IHC,ICC,IF,ELISA

Application Details: Western blot, 0.25-0.5 μg/ml, Human
Immunohistochemistry(Paraffin-embedded Section), 2-5 μg/ml, Human
Immunocytochemistry/Immunofluorescence, 5 μg/ml, Human
ELISA, 0.1-0.5 μg/ml, -

Gene Name: HSD3B7

Specificity:

Background: This gene encodes an enzyme which is involved in the initial stages of the synthesis of bile acids from cholesterol and a member of the short-chain dehydrogenase/reductase superfamily. The encoded protein is a membrane-associated endoplasmic reticulum protein which is active against 7-alpha hydrosylated sterol substrates. Mutations in this gene are associated with a congenital bile acid synthesis defect which leads to neonatal cholestasis, a form of progressive liver disease. Multiple transcript variants encoding different isoforms have been found for this gene.

Immunogen: E.coli-derived human HSD3B7 recombinant protein (Position: E23-A363). Human HSD3B7 shares 87.7% amino acid (aa) sequence identity with mouse HSD3B7.

Clonality: Polyclonal

Contents: Each vial contains 4 mg Trehalose, 0.9 mg NaCl, 0.2 mg Na2HPO4.

Purification: Immunogen affinity purified.

Reconstitution: Adding 0.2 ml of distilled water will yield a concentration of 500 μg/ml.

Reference: 1. Cheng, J. B., Jacquemin, E., Gerhardt, M., Nazer, H., Cresteil, D., Heubi, J. E., Setchell, K. D. R., Russell, D. W. Molecular genetics of 3-beta-hydroxy-D5-C27-steroid oxidoreductase deficiency in 16 patients with loss of bile acid synthesis and liver disease. J. Clin. Endocr. Metab. 88: 1833-1841, 2003. 2. Clayton, P. T., Leonard, J. V., Lawson, A. M., Setchell, K. D. R., Andersson, S., Egestad, B., Sjovall, J. Familial giant cell hepatitis associated with synthesis of 3-beta,7-alpha-dihydroxy- and 3-beta,7-alpha,12-alpha-trihydroxy-5-cholenoic acids. J. Clin. Invest. 79: 1031-1038, 1987. 3. Schwarz, M., Wright, A. C., Davis, D. L., Nazer, H., Bjorkhem, I., Russell, D. W. The bile acid synthetic gene 3-beta-hydroxy-delta-5-C27-steroid oxidoreductase is mutated in progressive intrahepatic cholestasis. J. Clin. Invest. 106: 1175-1184, 2000.

Uniprot ID: Q9H2F3

Host: Rabbit

Concentration: Adding 0.2 ml of distilled water will yield a concentration of 500 μg/ml.

Conjugate:

Cross Reactivity: No cross reactivity with other proteins.

Isotype: IgG

Phospho_site:

Clone Number:

Observed Molecular Weight: 45 kDa

Calculated Molecular Weight: 52588 MW

Gene ID: 80270

Protein Name: 3 beta-hydroxysteroid dehydrogenase type 7

Gene Full Name: hydroxy-delta-5-steroid dehydrogenase, 3 beta- and steroid delta-isomerase 7

Synonyms: PFIC4; SDR11E3