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Rabbit Anti-HSD3B7  antibody (bs-2366R)  
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產品編號 bs-2366R
英文名稱 Rabbit Anti-HSD3B7  antibody
中文名稱 滋養(yǎng)層細胞抗原3β7抗體
別    名 3 beta HSD VII; 3 beta hydroxy Delta(5) C27 steroid oxidoreductase; 3 beta hydroxysteroid dehydrogenase type 7; 3 beta hydroxysteroid dehydrogenase type VII; C(27) 3 beta HSD; Cca2; Cholest 5 ene 3 beta,7 alpha diol 3 beta dehydrogenase; Confluent 3Y1 cell associated 2; Hydroxy delta 5 steroid dehydrogenase, 3 beta and steroid delta isomerase 7; OTTMUSP00000018894; PFIC4; SDR11E3; BB098564; OTTMUSP00000018895; OTTMUSP00000018897; AI195443.  
研究領域 腫瘤  細胞生物  免疫學  內皮細胞  
抗體來源 Rabbit
克隆類型 Polyclonal
交叉反應 Human,Rat (predicted: Mouse,Rabbit,Pig,Cow)
產品應用 WB=1:500-2000,IHC-P=1:100-500,IHC-F=1:100-500,IF=1:100-500,ELISA=1:5000-10000
not yet tested in other applications.
optimal dilutions/concentrations should be determined by the end user.
理論分子量 41kDa
細胞定位 細胞漿 細胞膜 
性    狀 Liquid
濃    度 1mg/ml
免 疫 原 KLH conjugated synthetic peptide derived from human HSD3B7: 101-200/369 
亞    型 IgG
純化方法 affinity purified by Protein A
緩 沖 液 0.01M TBS (pH7.4) with 1% BSA, 0.02% Proclin300 and 50% Glycerol.
保存條件 Shipped at 4℃. Store at -20℃ for one year. Avoid repeated freeze/thaw cycles.
注意事項 This product as supplied is intended for research use only, not for use in human, therapeutic or diagnostic applications.
PubMed PubMed
產品介紹 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].

Function:
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.

Subcellular Location:
Endoplasmic reticulum membrane; Multi-pass membrane protein.

Tissue Specificity:
High levels in liver and lung, moderate levels in spleen, brain, heart, kidney, jejunum and testis. Up-regulated in 3Y1 cells upon growth arrest.

DISEASE:
Defects in HSD3B7 are the cause of congenital bile acid synthesis defect type 1 (CBAS1) [MIM:607765]; 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.

Similarity:
Belongs to the 3-beta-HSD family.

SWISS:
Q9H2F3

Gene ID:
80270

Database links:

Entrez Gene: 80270 Human

Entrez Gene: 101502 Mouse

Omim: 607764 Human

SwissProt: Q9H2F3 Human

SwissProt: Q9EQC1 Mouse



產品圖片
Sample: Lane 1: Human HeLa cell lysates Lane 2: Human HepG2 cell lysates Lane 3: Human MCF-7 cell lysates Lane 4: Human A549 cell lysates Primary: Anti-HSD3B7 (bs-2366R) at 1/1000 dilution Secondary: IRDye800CW Goat Anti-Rabbit IgG at 1/20000 dilution Predicted band size: 41 kDa Observed band size: 45 kDa
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