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    NBEAL2 Rabbit pAb (bs-19031R)  
    訂購熱線:400-901-9800
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    50ul/1180.00元
    100ul/1980.00元
    200ul/2800.00元
    大包裝/詢價
    產品編號 bs-19031R
    英文名稱 NBEAL2 Rabbit pAb
    中文名稱 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.  
    研究領域 細胞生物  發育生物學  
    抗體來源 Rabbit
    克隆類型 Polyclonal
    克 隆 號
    交叉反應 (predicted: Human,Mouse,Rat,Rabbit,Pig,Horse)
    產品應用 WB=1:500-2000,IHC-P=1:100-500,IHC-F=1:100-500,IF=1:100-500,ICC/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 303 kDa
    檢測分子量
    細胞定位 細胞漿 
    性    狀 Liquid
    濃    度 1mg/ml
    免 疫 原 KLH conjugated synthetic peptide derived from human NBEAL2: 101-200/2754 
    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 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.

    SWISS:
    Q6ZNJ1

    Gene ID:
    23218

    Database links:

    Entrez Gene: 23218 Human

    Omim: 614169 Human

    SwissProt: Q6ZNJ1 Human

    Unigene: 437043 Human



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