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Rabbit Anti-STXBP2/BF555 Conjugated antibody (bs-12837R-BF555)
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說(shuō) 明 書: 100ul  
100ul/2980.00元
大包裝/詢價(jià)
產(chǎn)品編號(hào) bs-12837R-BF555
英文名稱 Rabbit Anti-STXBP2/BF555 Conjugated antibody
中文名稱 BF555標(biāo)記的突觸融合蛋白結(jié)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(guī)格價(jià)格 100ul/2980元 購(gòu)買        大包裝/詢價(jià)
說(shuō) 明 書 100ul  
研究領(lǐng)域 細(xì)胞生物  神經(jīng)生物學(xué)  信號(hào)轉(zhuǎn)導(dǎo)  結(jié)合蛋白  
抗體來(lái)源 Rabbit
克隆類型 Polyclonal
交叉反應(yīng) (predicted: Human, Mouse, Rat, Pig, Horse, )
產(chǎn)品應(yīng)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 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(chǔ) 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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