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    Rabbit Anti-CYP11A1/BF488 Conjugated antibody (bs-3608R-BF488)
    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  
    100ul/2980.00元
    大包裝/詢(xún)價(jià)
    產(chǎn)品編號 bs-3608R-BF488
    英文名稱(chēng) Rabbit Anti-CYP11A1/BF488 Conjugated antibody
    中文名稱(chēng) BF488標記的細胞色素P450 11A1抗體
    別    名 Cholesterol 20 22 desmolase; Cholesterol desmolase; Cholesterol monooxygenase (side chain cleaving); Cholesterol side chain cleavage enzyme; Cholesterol side chain cleavage enzyme mitochondrial; Cholesterol side-chain cleavage enzyme; CP11A_HUMAN; CYP11A; CYP11A1; CYPXIA1; Cytochrome P450 11A1; Cytochrome P450 11A1 mitochondrial; Cytochrome P450 family 11 subfamily A polypeptide 1; Cytochrome P450 subfamily XIA; Cytochrome P450(scc); Cytochrome P450C11A1; mitochondrial; P450SCC; Steroid 20 22 lyase.  
    規格價(jià)格 100ul/2980元 購買(mǎi)        大包裝/詢(xún)價(jià)
    說(shuō) 明 書(shū) 100ul  
    研究領(lǐng)域 腫瘤  細胞生物  免疫學(xué)  轉錄調節因子  線(xiàn)粒體  
    抗體來(lái)源 Rabbit
    克隆類(lèi)型 Polyclonal
    交叉反應 Mouse, Rat,  (predicted: Human, )
    產(chǎn)品應用 IF=1:50-200 
    not yet tested in other applications.
    optimal dilutions/concentrations should be determined by the end user.
    分 子 量 53/57kDa
    性    狀 Lyophilized or Liquid
    濃    度 1mg/ml
    免 疫 原 KLH conjugated synthetic peptide derived from human CYP11A1/P450SCC
    亞    型 IgG
    純化方法 affinity purified by Protein A
    儲 存 液 0.01M TBS(pH7.4) with 1% BSA, 0.03% Proclin300 and 50% Glycerol
    保存條件 Store at -20 °C for one year. Avoid repeated freeze/thaw cycles. The lyophilized antibody is stable at room temperature for at least one month and for greater than a year when kept at -20°C. When reconstituted in sterile pH 7.4 0.01M PBS or diluent of antibody the antibody is stable for at least two weeks at 2-4 °C.
    產(chǎn)品介紹 background:
    This gene encodes a member of the cytochrome P450 superfamily of enzymes. The cytochrome P450 proteins are monooxygenases which catalyze many reactions involved in drug metabolism and synthesis of cholesterol, steroids and other lipids. This protein localizes to the mitochondrial inner membrane and catalyzes the conversion of cholesterol to pregnenolone, the first and rate-limiting step in the synthesis of the steroid hormones. Two transcript variants encoding different isoforms have been found for this gene. The cellular location of the smaller isoform is unclear since it lacks the mitochondrial-targeting transit peptide. [provided by RefSeq, Jul 2008]

    Function:
    Catalyzes the side-chain cleavage reaction of cholesterol to pregnenolone.

    Subcellular Location:
    Mitochondrion membrane.

    DISEASE:
    Defects in CYP11A1 are the cause of adrenal insufficiency congenital with 46,XY sex reversal (AICSR) [MIM:613743]. A rare disorder that can present as acute adrenal insufficiency in infancy or childhood. ACTH and plasma renin activity are elevated and adrenal steroids are inappropriately low or absent; the 46,XY patients have female external genitalia, sometimes with clitoromegaly. The phenotypic spectrum ranges from prematurity, complete underandrogenization, and severe early-onset adrenal failure to term birth with clitoromegaly and later-onset adrenal failure. Patients with congenital adrenal insufficiency do not manifest the massive adrenal enlargement typical of congenital lipoid adrenal hyperplasia.

    Similarity:
    Belongs to the cytochrome P450 family.

    Database links:

    Entrez Gene: 1583 Human

    Omim: 118485 Human

    SwissProt: P05108 Human

    Unigene: 303980 Human



    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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