Anti-HSD3B7 Antibody (CAB17777)
- SKU:
- CAB17777
- Product type:
- Antibody
- Reactivity:
- Mouse
- Reactivity:
- Rat
- Host Species:
- Rabbit
- Isotype:
- IgG
- Antibody Type:
- Polyclonal Antibody
Frequently bought together:
Description
| 抗体名: | Anti-HSD3B7 Antibody |
| 抗体コード: | CAB17777 |
| 抗体サイズ: | 20uL, 50uL, 100uL |
| 申し込み: | WB |
| 反応性: | Mouse, Rat |
| 宿主種: | Rabbit |
| 免疫原: | Recombinant fusion protein containing a sequence corresponding to amino acids 120-280 of human HSD3B7 (NP_079469.2). |
| 申し込み: | WB |
| 推奨希釈: | WB 1:500 - 1:2000 |
| 反応性: | Mouse, Rat |
| ポジティブサンプル: | mouse brain |
| 免疫原: | Recombinant fusion protein containing a sequence corresponding to amino acids 120-280 of human HSD3B7 (NP_079469.2). |
| 精製方法: | Affinity purification |
| ストレージバッファ: | Store at -20°C. Avoid freeze / thaw cycles. Buffer: PBS with 0.02% sodium azide, 50% glycerol, pH7.3. |
| アイソタイプ: | IgG |
| 順序: | GTRF LVYT SSME VVGP NTKG HPFY RGNE DTPY EAVH RHPY PCSK ALAE WLVL EANG RKVR GGLP LVTC ALRP TGIY GEGH QIMR DFYR QGLR LGGW LFRA IPAS VEHG RVYV GNVA WMHV LAAR ELEQ RATL MGGQ VYFC YDGS PYRS YEDF NMEF LGPC G |
| 遺伝子ID: | 80270 |
| Uniprot: | Q9H2F3 |
| セルラーロケーション: | |
| 計算された分子量: | 41kDa |
| 観察された分子量: | 41kDa |
 | Western blot - HSD3B7 Polyclonal Antibody (CAB17777) |
| 同義語: | CBAS1, PFIC4, SDR11E3, HSD3B7 |
| バックグラウンド: | 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. |
| UniProt Protein Function: | HSD3B7: The 3-beta-HSD enzymatic system plays a crucial role in the biosynthesis of all classes of hormonal steroids. HSD VII is active against four 7-alpha-hydroxylated sterols. Does not metabolize several different C(19/21) steroids as substrates. Involved in bile acid synthesis. Defects in HSD3B7 are the cause of congenital bile acid synthesis defect type 1 (CBAS1); also known as neonatal progressive intrahepatic cholestasis. CBAS1 is due to a primary defect in bile synthesis leading to progressive liver disease. Clinical features include neonatal jaundice, severe intrahepatic cholestasis and cirrhosis. Belongs to the 3-beta-HSD family. 2 isoforms of the human protein are produced by alternative splicing. |
| UniProt Protein Details: | Protein type:Membrane protein, multi-pass; Membrane protein, integral; EC 1.1.1.181; Lipid Metabolism - primary bile acid biosynthesis; Oxidoreductase Chromosomal Location of Human Ortholog: 16p11.2 Cellular Component: endoplasmic reticulum membrane; integral to membrane Molecular Function:3-beta-hydroxy-delta5-steroid dehydrogenase activity; cholest-5-ene-3-beta,7-alpha-diol 3-beta-dehydrogenase activity; protein binding Biological Process: bile acid biosynthetic process; bile acid metabolic process Disease: Bile Acid Synthesis Defect, Congenital, 1 |
| NCBI Summary: | 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. [provided by RefSeq, Dec 2008] |
| UniProt Code: | Q9H2F3 |
| NCBI GenInfo Identifier: | 47605550 |
| NCBI Gene ID: | 80270 |
| NCBI Accession: | Q9H2F3.2 |
| UniProt Secondary Accession: | Q9H2F3,Q96M28, Q9BSN9, |
| UniProt Related Accession: | Q9H2F3 |
| Molecular Weight: | 21,322 Da |
| NCBI Full Name: | 3 beta-hydroxysteroid dehydrogenase type 7 |
| NCBI Synonym Full Names: | hydroxy-delta-5-steroid dehydrogenase, 3 beta- and steroid delta-isomerase 7 |
| NCBI Official Symbol: | HSD3B7 |
| NCBI Official Synonym Symbols: | CBAS1; PFIC4; SDR11E3 |
| NCBI Protein Information: | 3 beta-hydroxysteroid dehydrogenase type 7 |
| UniProt Protein Name: | 3 beta-hydroxysteroid dehydrogenase type 7 |
| UniProt Synonym Protein Names: | 3 beta-hydroxysteroid dehydrogenase type VII; 3-beta-HSD VII; 3-beta-hydroxy-Delta(5)-C27 steroid oxidoreductase (EC:1.1.1.-); C(27) 3-beta-HSD; Cholest-5-ene-3-beta,7-alpha-diol 3-beta-dehydrogenase (EC:1.1.1.181) |
| Protein Family: | 3 beta-hydroxysteroid dehydrogenase |
| UniProt Gene Name: | HSD3B7 |
| UniProt Entry Name: | 3BHS7_HUMAN |