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    Rabbit Anti-NBEAL2/Cy3 Conjugated antibody (bs-19031R-Cy3)
    訂購熱線:400-901-9800
    訂購郵箱:sales@bioss.com.cn
    訂購QQ:  400-901-9800
    技術支持:techsupport@bioss.com.cn
    說 明 書: 100ul  
    100ul/2980.00元
    大包裝/詢價
    產品編號 bs-19031R-Cy3
    英文名稱 Rabbit Anti-NBEAL2/Cy3 Conjugated antibody
    中文名稱 Cy3標記的蛋白激酶錨定蛋白樣蛋白2抗體
    別    名 BDPLT4; GPS; NBEAL2; NBEL2_HUMAN; Neurobeachin-like protein 2; UNQ253/PRO290.  
    規格價格 100ul/2980元 購買        大包裝/詢價
    說 明 書 100ul  
    研究領域 細胞生物  發育生物學  
    抗體來源 Rabbit
    克隆類型 Polyclonal
    交叉反應 (predicted: Human, Mouse, Rat, Pig, Horse, Rabbit, )
    產品應用 ICC=1:50-200 IF=1:50-200 
    not yet tested in other applications.
    optimal dilutions/concentrations should be determined by the end user.
    分 子 量 303kDa
    性    狀 Lyophilized or Liquid
    濃    度 1mg/ml
    免 疫 原 KLH conjugated synthetic peptide derived from human NBEAL2
    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.
    產品介紹 background:
    The protein encoded by this gene contains a beige and Chediak-Higashi (BEACH) domain and multiple WD40 domains, and may play a role in megakaryocyte alpha-granule biogenesis. Mutations in this gene are a cause of gray platelet syndrome. [provided by RefSeq, Dec 2011]

    Function:
    Probably involved in thrombopoiesis. Plays a role in the development or secretion of alpha-granules, that contain several growth factors important for platelet biogenesis.

    Subcellular Location:
    Endoplasmic reticulum.

    Tissue Specificity:
    Expressed in megakaryocytes.

    DISEASE:
    Gray platelet syndrome (GPS) [MIM:139090]: A rare platelet disorder characterized by a selective deficiency in the number and contents of platelet alpha-granules. It is associated with mild to moderate bleeding tendency and moderate thrombocytopenia. The platelets are enlarged and have a gray appearance on light microscopy of Wright-stained peripheral blood smears due to decreased granules.

    Similarity:
    Belongs to the WD repeat neurobeachin family.
    Contains 1 BEACH domain.
    Contains 5 WD repeats.

    Database links:

    Entrez Gene: 23218 Human

    Omim: 614169 Human

    SwissProt: Q6ZNJ1 Human

    Unigene: 437043 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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