{"product_id":"anti-hsd3b7-antibody-picoband-r","title":"Anti-HSD3B7 Antibody Picoband®","description":"\u003cp\u003e\u003cb\u003eSize\u003c\/b\u003e: 100 μg\u003c\/p\u003e\u003cp\u003e\u003cb\u003eStorage\u003c\/b\u003e: At -20℃ for one year from date of receipt. \nAfter reconstitution, at 4℃ for one month. It can also be aliquotted and stored frozen at -20℃ for six months.\nAvoid repeated freezing and thawing.\u003c\/p\u003e\u003cp\u003e\u003cb\u003eForm\u003c\/b\u003e: Lyophilized\u003c\/p\u003e\u003cp\u003e\u003cb\u003eReactivity\u003c\/b\u003e: Human\u003c\/p\u003e\u003cp\u003e\u003cb\u003eApplications\u003c\/b\u003e: WB,IHC,ICC,IF,ELISA\u003c\/p\u003e\u003cp\u003e\u003cb\u003eApplication Details\u003c\/b\u003e: Western blot, 0.25-0.5 μg\/ml, Human\u003cbr\u003e\nImmunohistochemistry(Paraffin-embedded Section), 2-5 μg\/ml, Human\u003cbr\u003e\nImmunocytochemistry\/Immunofluorescence, 5 μg\/ml, Human\u003cbr\u003e\nELISA, 0.1-0.5 μg\/ml, -\u003cbr\u003e\u003c\/p\u003e\u003cp\u003e\u003cb\u003eGene Name\u003c\/b\u003e: HSD3B7\u003c\/p\u003e\u003cp\u003e\u003cb\u003eSpecificity\u003c\/b\u003e: \u003c\/p\u003e\u003cp\u003e\u003cb\u003eBackground\u003c\/b\u003e: 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.\u003c\/p\u003e\u003cp\u003e\u003cb\u003eImmunogen\u003c\/b\u003e: E.coli-derived human HSD3B7 recombinant protein (Position: E23-A363). Human HSD3B7 shares 87.7% amino acid (aa) sequence identity with mouse HSD3B7.\u003c\/p\u003e\u003cp\u003e\u003cb\u003eClonality\u003c\/b\u003e: Polyclonal\u003c\/p\u003e\u003cp\u003e\u003cb\u003eContents\u003c\/b\u003e: Each vial contains 4 mg Trehalose, 0.9 mg NaCl, 0.2 mg Na2HPO4.\u003c\/p\u003e\u003cp\u003e\u003cb\u003ePurification\u003c\/b\u003e: Immunogen affinity purified.\u003c\/p\u003e\u003cp\u003e\u003cb\u003eReconstitution\u003c\/b\u003e: Adding 0.2 ml of distilled water will yield a concentration of 500 μg\/ml.\u003c\/p\u003e\u003cp\u003e\u003cb\u003eReference\u003c\/b\u003e: 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.\n2. 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.\n3. 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.\u003c\/p\u003e\u003cp\u003e\u003cb\u003eUniprot ID\u003c\/b\u003e: Q9H2F3\u003c\/p\u003e\u003cp\u003e\u003cb\u003eHost\u003c\/b\u003e: Rabbit\u003c\/p\u003e\u003cp\u003e\u003cb\u003eConcentration\u003c\/b\u003e: Adding 0.2 ml of distilled water will yield a concentration of 500 μg\/ml.\u003c\/p\u003e\u003cp\u003e\u003cb\u003eConjugate\u003c\/b\u003e: \u003c\/p\u003e\u003cp\u003e\u003cb\u003eCross Reactivity\u003c\/b\u003e: No cross reactivity with other proteins.\u003c\/p\u003e\u003cp\u003e\u003cb\u003eIsotype\u003c\/b\u003e: IgG\u003c\/p\u003e\u003cp\u003e\u003cb\u003ePhospho_site\u003c\/b\u003e: \u003c\/p\u003e\u003cp\u003e\u003cb\u003eClone Number\u003c\/b\u003e: \u003c\/p\u003e\u003cp\u003e\u003cb\u003eObserved Molecular Weight\u003c\/b\u003e: 45 kDa\u003c\/p\u003e\u003cp\u003e\u003cb\u003eCalculated Molecular Weight\u003c\/b\u003e: 52588 MW\u003c\/p\u003e\u003cp\u003e\u003cb\u003eGene ID\u003c\/b\u003e: 80270\u003c\/p\u003e\u003cp\u003e\u003cb\u003eProtein Name\u003c\/b\u003e: 3 beta-hydroxysteroid dehydrogenase type 7\u003c\/p\u003e\u003cp\u003e\u003cb\u003eGene Full Name\u003c\/b\u003e: hydroxy-delta-5-steroid dehydrogenase, 3 beta- and steroid delta-isomerase 7\u003c\/p\u003e\u003cp\u003e\u003cb\u003eSynonyms\u003c\/b\u003e: PFIC4; SDR11E3\u003c\/p\u003e","brand":"GeneBio Systems","offers":[{"title":"Default Title","offer_id":48787953320036,"sku":"A10025-1","price":506.95,"currency_code":"EUR","in_stock":true}],"thumbnail_url":"\/\/cdn.shopify.com\/s\/files\/1\/0558\/8588\/9636\/files\/no_image_default_image-jpeg_31b87f82-8058-4e1c-bbdf-2f675694e425.jpg?v=1784405920","url":"https:\/\/www.genebiosystems.com\/en-fr\/products\/anti-hsd3b7-antibody-picoband-r","provider":"GeneBio ","version":"1.0","type":"link"}