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Rabbit Anti-HSD3B7/Gold Conjugated antibody (bs-2366R-Gold)
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說 明 書: 100ul(10nm  15nm  35nm
100ul/2980.00元
大包裝/詢價
產品編號 bs-2366R-Gold
英文名稱1 Rabbit Anti-HSD3B7/Gold Conjugated antibody
中文名稱 膠體金標記的滋養層細胞抗原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.  
規格價格 100ul/2980元 購買        大包裝/詢價
說 明 書 100ul(10nm  15nm  35nm
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 (predicted: Human, Mouse, Rat, Pig, Cow, Rabbit, )
產品應用 IEM=1:20-200 ICA=1:20-200 ChIP=1:20-200 
not yet tested in other applications.
optimal dilutions/concentrations should be determined by the end user.
分 子 量 41kDa
性    狀 Lyophilized or Liquid
濃    度 0.4mg/ml
免 疫 原 KLH conjugated synthetic peptide derived from human HSD3B7 (301-369aa)
亞    型 IgG
純化方法 affinity purified by Protein A
儲 存 液 0.02M TBS(pH8.2) with 1% BSA, 0.03% Proclin300.
保存條件 Store at 2-8 oC for 3-6 months. Avoid repeated freeze/thaw cycles.
產品介紹 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. [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.

Database links:

Entrez Gene: 80270 Human

Entrez Gene: 101502 Mouse

Omim: 607764 Human

SwissProt: Q9H2F3 Human

SwissProt: Q9EQC1 Mouse



Important Note:
This product as supplied is intended for research use only, not for use in human, therapeutic or diagnostic applications.
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