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Mouse Anti-human CD127-APC/BF350 Conjugated antibody (bsm-30089M-APC-BF350)
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說 明 書: 100ul  
100ul/2980.00元
大包裝/詢價(jià)
產(chǎn)品編號 bsm-30089M-APC-BF350
英文名稱 Mouse Anti-human CD127-APC/BF350 Conjugated antibody
中文名稱 BF350標(biāo)記的APC標(biāo)記小鼠抗人CD127單克隆抗體
別    名 CD 127; CD127; CD127 antigen; CDW127; IL 7R alpha; IL 7R; IL-7 receptor subunit alpha; IL-7R subunit alpha; IL-7R-alpha; IL-7RA; IL7R; IL7RA; IL7RA_HUMAN; IL7Ralpha; ILRA; Interleukin 7 receptor alpha chain; Interleukin 7 receptor; Interleukin 7 receptor isoform H5 6; Interleukin-7 receptor subunit alpha.  
規(guī)格價(jià)格 100ul/2980元 購買        大包裝/詢價(jià)
說 明 書 100ul  
研究領(lǐng)域 細(xì)胞生物  免疫學(xué)  發(fā)育生物學(xué)  干細(xì)胞  淋巴細(xì)胞  t-淋巴細(xì)胞  b-淋巴細(xì)胞  
抗體來源 Mouse
克隆類型 Monoclonal
交叉反應(yīng) Human, 
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 50kDa
性    狀 Liquid
濃    度 1mg/ml
免 疫 原
亞    型 IgG
純化方法 affinity purified by Protein G
儲 存 液 Preservative: 15mM Sodium Azide, Constituents: 1% BSA, 0.01M PBS, pH 7.4.
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:
The protein encoded by this gene is a receptor for interleukine 7 (IL7). The function of this receptor requires the interleukin 2 receptor, gamma chain (IL2RG), which is a common gamma chain shared by the receptors of various cytokines, including interleukine 2, 4, 7, 9, and 15. This protein has been shown to play a critical role in the V(D)J recombination during lymphocyte development. This protein is also found to control the accessibility of the TCR gamma locus by STAT5 and histone acetylation. Knockout studies in mice suggested that blocking apoptosis is an essential function of this protein during differentiation and activation of T lymphocytes. The functional defects in this protein may be associated with the pathogenesis of the severe combined immunodeficiency (SCID).

Function:
Receptor for interleukin-7. Also acts as a receptor for thymic stromal lymphopoietin (TSLP).

Subcellular Location:
Secreted and Cell membrane.

Post-translational modifications:
N-glycosylated IL-7Ralpha binds IL7 300-fold more tightly than the unglycosylated form.

DISEASE:
Defects in IL7R are a cause of severe combined immunodeficiency autosomal recessive T-cell-negative/B-cell-positive/NK-cell-positive (T(-)B(+)NK(+) SCID) [MIM:608971]. A form of severe combined immunodeficiency (SCID), a genetically and clinically heterogeneous group of rare congenital disorders characterized by impairment of both humoral and cell-mediated immunity, leukopenia, and low or absent antibody levels. Patients present in infancy recurrent, persistent infections by opportunistic organisms. The common characteristic of all types of SCID is absence of T-cell-mediated cellular immunity due to a defect in T-cell development.
Genetic variations in IL7R are a cause of susceptibility to multiple sclerosis type 3 (MS3) [MIM:612595]. A multifactorial, inflammatory, demyelinating disease of the central nervous system. Sclerotic lesions are characterized by perivascular infiltration of monocytes and lymphocytes and appear as indurated areas in pathologic specimens (sclerosis in plaques). The pathological mechanism is regarded as an autoimmune attack of the myelin sheat, mediated by both cellular and humoral immunity. Clinical manifestations include visual loss, extra-ocular movement disorders, paresthesias, loss of sensation, weakness, dysarthria, spasticity, ataxia and bladder dysfunction. Genetic and environmental factors influence susceptibility to the disease. Note=A polymorphism at position 244 strongly influences susceptibility to multiple sclerosis. Overtransmission of the major 'C' allele coding for Thr-244 is detected in offspring affected with multiple sclerosis. In vitro analysis of transcripts from minigenes containing either 'C' allele (Thr-244) or 'T' allele (Ile-244) shows that the 'C' allele results in an approximately two-fold increase in the skipping of exon 6, leading to increased production of a soluble form of IL7R. Thus, the multiple sclerosis associated 'C' risk allele of IL7R would probably decrease membrane-bound expression of IL7R. As this risk allele is common in the general population, some additional triggers are probably required for the development and progression of MS.

Similarity:
Belongs to the type I cytokine receptor family. Type 4 subfamily.
Contains 1 fibronectin type-III domain.

Database links:

Entrez Gene: 3575 Human

Omim: 146661 Human

SwissProt: P16871 Human

Unigene: 591742 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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