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    Rabbit Anti-HSD3B7/Gold Conjugated antibody (bs-2366R-Gold)
    訂購熱線(xiàn):400-901-9800
    訂購郵箱:sales@bioss.com.cn
    訂購QQ:  400-901-9800
    技術(shù)支持:techsupport@bioss.com.cn
    說(shuō) 明 書(shū): 100ul(10nm  15nm  35nm
    100ul/2980.00元
    大包裝/詢(xún)價(jià)
    產(chǎn)品編號 bs-2366R-Gold
    英文名稱(chēng) Rabbit Anti-HSD3B7/Gold Conjugated antibody
    中文名稱(chēng) 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(jià)格 100ul/2980元 購買(mǎi)        大包裝/詢(xún)價(jià)
    說(shuō) 明 書(shū) 100ul(10nm  15nm  35nm
    研究領(lǐng)域 腫瘤  細胞生物  免疫學(xué)  內皮細胞  
    抗體來(lái)源 Rabbit
    克隆類(lèi)型 Polyclonal
    交叉反應 (predicted: Human, Mouse, Rat, Pig, Cow, Rabbit, )
    產(chǎn)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(chǎn)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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