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Rabbit Anti-IQSEC2/BF647 Conjugated antibody (bs-13645R-BF647)
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說(shuō) 明 書(shū): 100ul  
100ul/2980.00元
大包裝/詢價(jià)
產(chǎn)品編號(hào) bs-13645R-BF647
英文名稱 Rabbit Anti-IQSEC2/BF647 Conjugated antibody
中文名稱 BF647標(biāo)記的IQSEC2抗體
別    名 BRAG1; IQ motif and SEC7 domain-containing protein 2; IQEC2_HUMAN; Iqsec2; KIAA0522; mKIAA0522; MRX1; RP11-258C19.1.  
規(guī)格價(jià)格 100ul/2980元 購(gòu)買        大包裝/詢價(jià)
說(shuō) 明 書(shū) 100ul  
研究領(lǐng)域 腫瘤  細(xì)胞生物  神經(jīng)生物學(xué)  信號(hào)轉(zhuǎn)導(dǎo)  細(xì)胞骨架  
抗體來(lái)源 Rabbit
克隆類型 Polyclonal
交叉反應(yīng) Mouse,  (predicted: Human, Rat, Dog, Pig, Cow, Sheep, )
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 162kDa
性    狀 Lyophilized or Liquid
濃    度 1mg/ml
免 疫 原 KLH conjugated synthetic peptide derived from human IQSEC2
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:
IQSEC2 is a 1,478 amino acid protein that belongs to the BRAG family and contains one IQ domain, one PH domain and a SEC7 domain. Localizing to the cytoplasm, IQSEC2 is expressed in brain, kidney and small intestine, with weaker levels of expression in placenta, pancreas, ovary, prostate and liver. IQSEC2 is a component of the postsynaptic density at excitatory synapses, and interacts with ARF family members as a guanine nucleotide exchange factor. Through the activation of ARF substrates, IQSEC2 may play a crucial role in cytoskeletal and synaptic organization. The gene encoding IQSEC2 maps to the human X chromosome. Defects to the IQSEC2 gene have been linked to mental retardation X-linked type 1 (MRX1), a condition characterized by decreased intellectual function. IQSEC2 exists as three isoforms due to alternative splicing events.

Subcellular Location:
Cytoplasm.

Tissue Specificity:
Expressed in brain, kidney and small intestine. Weakly expressed in placenta, pancreas, ovary, prostate and liver.

DISEASE:
Defects in IQSEC2 are the cause of mental retardation X-linked type 1 (MRX1) [MIM:309530]. Mental retardation is characterized by significantly below average general intellectual functioning associated with impairments in adaptative behavior and manifested during the developmental period. In contrast to syndromic or specific X-linked mental retardation which also present with associated physical, neurological and/or psychiatric manifestations, intellectual deficiency is the only primary symptom of non-syndromic X-linked mental retardation.

Similarity:
Belongs to the BRAG family.
Contains 1 IQ domain.
Contains 1 PH domain.
Contains 1 SEC7 domain.

Database links:

Entrez Gene: 23096 Human

Omim: 300522 Human

SwissProt: Q5JU85 Human

Unigene: 496138 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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