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    Rabbit Anti-STXBP2/AP Conjugated antibody (bs-12837R-AP)
    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-12837R-AP
    英文名稱(chēng) Rabbit Anti-STXBP2/AP Conjugated antibody
    中文名稱(chēng) 堿性磷酸酶(AP)標記的突觸融合蛋白結合蛋白2抗體
    別    名 FHL5; Hunc18b; MUNC18 2; pp10122; protein unc-18 homolog 2; protein unc-18 homolog B; STXB2_HUMAN; Stxbp2; syntaxin binding protein 2; Syntaxin-binding protein 2; Unc-18B; UNC18 2; Unc18-2; UNC18B.  
    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 細胞生物  神經(jīng)生物學(xué)  信號轉導  結合蛋白  
    抗體來(lái)源 Rabbit
    克隆類(lèi)型 Polyclonal
    交叉反應 (predicted: Human, Mouse, Rat, Pig, Horse, )
    產(chǎn)品應用 IHC-P=1:50-200 IHC-F=1:50-200 ICC=1:50-200 
    not yet tested in other applications.
    optimal dilutions/concentrations should be determined by the end user.
    分 子 量 66kDa
    性    狀 Lyophilized or Liquid
    濃    度 1mg/ml
    免 疫 原 KLH conjugated synthetic peptide derived from human STXBP2
    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 STXBP/unc-18/SEC1 family. The encoded protein is involved in intracellular trafficking, control of SNARE (soluble NSF attachment protein receptor) complex assembly, and the release of cytotoxic granules by natural killer cells. Mutations in this gene are associated with familial hemophagocytic lymphohistiocytosis. Alternatively spliced transcript variants encoding different isoforms have been noted for this gene. [provided by RefSeq, Jan 2013]

    Function:
    Involved in intracellular vesicle trafficking and vesicle fusion with membranes. Contributes to the granule exocytosis machinery through interaction with soluble N-ethylmaleimide-sensitive factor attachment protein receptor (SNARE) proteins that regulate membrane fusion. Regulates cytotoxic granule exocytosis in natural killer (NK) cells.

    Subunit:
    Interacts with STX1A, STX2 and STX3 (By similarity). Interacts with STX11.

    Tissue Specificity:
    Placenta, lung, liver, kidney and pancreas, as well as in peripheral blood lymphocytes.

    Similarity:
    Belongs to the STXBP/unc-18/SEC1 family.

    Database links:

    Entrez Gene: 515618 Cow

    Entrez Gene: 403880 Dog

    Entrez Gene: 6813 Human

    Entrez Gene: 20911 Mouse

    Entrez Gene: 81804 Rat

    Omim: 601717 Human

    SwissProt: Q28288 Dog

    SwissProt: Q15833 Human

    SwissProt: Q64324 Mouse

    SwissProt: Q62753 Rat

    Unigene: 515104 Human

    Unigene: 7247 Mouse

    Unigene: 10121 Rat



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

    Defects in STXBP2 are the cause of hemophagocytic lymphohistiocytosis familial type 5 (FHL5) [MIM:613101]. FHL5 is rare disorder characterized by immune dysregulation with hypercytokinemia, defective function of natural killer cell, and massive infiltration of several organs by activated lymphocytes and macrophages. The clinical features of the disease include fever, hepatosplenomegaly, cytopenia, and less frequently neurological abnormalities ranging from irritability and hypotonia to seizures, cranial nerve deficits and ataxia.
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