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  4. JAK3 Recombinant Monoclonal Antibody

JAK3 Recombinant Monoclonal Antibody

  • 货号：
    CSB-RA206737A0HU
  • 规格：
    ￥1320
  • 图片：
    • IHC image of CSB-RA206737A0HU diluted at 1:100 and staining in paraffin-embedded human lung cancer performed on a Leica BondTM system. After dewaxing and hydration, antigen retrieval was mediated by high pressure in a citrate buffer (pH 6.0). Section was blocked with 10% normal goat serum 30min at RT. Then primary antibody (1% BSA) was incubated at 4℃ overnight. The primary is detected by a Goat anti-rabbit IgG polymer labeled by HRP and visualized using 0.05% DAB.
  • 其他：

产品详情

  • 产品描述：

    JAK3 is only expressed in hematopoietic lineages and is exclusively coexpressed and linked with the gamma chain (γc). JAK3 is a key regulator of lymphopoiesis, and mutations in the JAK3 gene can cause severe combined immunodeficiency, as well as leukemia and lymphomas. JAK3 works in a heterodimeric pair with JAK1 in cytokine signaling, transmitting signals from IL-2, IL-4, IL-7, IL-9, IL-15, and IL-21 that govern lymphoid lineage cell growth and activation. JAK3 deficiency causes signal transduction through the common c to be disrupted, resulting in immunodeficiency, suggesting that targeted inhibition of JAK3 kinase may cause immunosuppression.

    The recombinant JAK3 antibody is a monoclonal antibody generated by cloning JAK3 antibody genes into plasma vectors and transfecting vector clones into stable cell lines for production. For recombinant antibody generation, mammalian cell lines like CHO cells and HEK293 are commonly used. The recombinant JAK3 antibody was purified using Affinity-chromatography. It has verified to detect JAK3 protein from Human in the ELISA, IHC.

  • Uniprot No.：
    P52333
  • 基因名：
  • 别名：
    Tyrosine-protein kinase JAK3 (EC 2.7.10.2) (Janus kinase 3) (JAK-3) (Leukocyte janus kinase) (L-JAK), JAK3
  • 反应种属：
    Human
  • 免疫原：
    A synthesized peptide derived from human JAK3
  • 免疫原种属：
    Homo sapiens (Human)
  • 标记方式：
    Non-conjugated
  • 克隆类型：
    Monoclonal
  • 抗体亚型：
    Rabbit IgG
  • 纯化方式：
    Affinity-chromatography
  • 克隆号：
    1C7
  • 浓度：
    It differs from different batches. Please contact us to confirm it.
  • 保存缓冲液：
    Rabbit IgG in phosphate buffered saline, pH 7.4, 150mM NaCl, 0.02% sodium azide and 50% glycerol.
  • 产品提供形式：
    Liquid
  • 应用范围：
    ELISA, IHC
  • 推荐稀释比：
    Application Recommended Dilution
    IHC 1:50-1:200
  • Protocols：
  • 储存条件：
    Upon receipt, store at -20°C or -80°C. Avoid repeated freeze.
  • 货期：
    Basically, we can dispatch the products out in 1-3 working days after receiving your orders. Delivery time maybe differs from different purchasing way or location, please kindly consult your local distributors for specific delivery time.

产品评价

靶点详情

  • 功能：
    Non-receptor tyrosine kinase involved in various processes such as cell growth, development, or differentiation. Mediates essential signaling events in both innate and adaptive immunity and plays a crucial role in hematopoiesis during T-cells development. In the cytoplasm, plays a pivotal role in signal transduction via its association with type I receptors sharing the common subunit gamma such as IL2R, IL4R, IL7R, IL9R, IL15R and IL21R. Following ligand binding to cell surface receptors, phosphorylates specific tyrosine residues on the cytoplasmic tails of the receptor, creating docking sites for STATs proteins. Subsequently, phosphorylates the STATs proteins once they are recruited to the receptor. Phosphorylated STATs then form homodimer or heterodimers and translocate to the nucleus to activate gene transcription. For example, upon IL2R activation by IL2, JAK1 and JAK3 molecules bind to IL2R beta (IL2RB) and gamma chain (IL2RG) subunits inducing the tyrosine phosphorylation of both receptor subunits on their cytoplasmic domain. Then, STAT5A AND STAT5B are recruited, phosphorylated and activated by JAK1 and JAK3. Once activated, dimerized STAT5 translocates to the nucleus and promotes the transcription of specific target genes in a cytokine-specific fashion.
  • 基因功能参考文献：
    1. JAK3 mutations in Italian patients affected by SCID: New molecular aspects of a long-known gene. PMID: 30032486
    2. we report the results of a screening for mutations in SETBP1 and JAK3 of a cohort of seventy Italian patients with juvenile myelomonocytic leukemia, identifying 11.4% of them harboring secondary mutations in these two genes and discovering two new mutations in the SKI domain of SETBP1 PMID: 26980750
    3. Jak3-mediated phosphorylation of beta-catenin suppressed EGF-mediated epithelial-mesenchymal transition and facilitated epithelial barrier functions by AJ localization of phosphorylated beta-catenin through its interactions with alpha-catenin. PMID: 28821617
    4. frequency of JAK3 mutations in the JH2 domain was relatively low in extranodal natural killer/T-cell lymphoma, nasal type (NTCL) in contrast to a previous report; study identified novel JAK3H583Y- and JAK3G589D-activating mutations that were oncogenic and sensitive to a JAK3 inhibitor PMID: 28284718
    5. In natural killer/T-cell lymphoma (NKTL) as a disease model, phosphorylation of EZH2 by JAK3 promotes the dissociation of the PRC2 complex leading to decreased global histone H3 lysine 27 methylation levels. PMID: 27297789
    6. JAK3 mediates smooth muscle cell proliferation and survival during injury-induced vascular remodeling. PMID: 28473442
    7. Data indicate that phosphorylation of Janus kinase 3 (JAK3) and STAT3 transcription factor (STAT3) was inhibited by latent membrane protein 1 (LMP1)-IgG. PMID: 28009988
    8. analysis of JAK3 kinetic mechanism and inhibition by tofacitinib PMID: 27555492
    9. patient had a homozygote of the JAK3 mutation, and her parents were heterozygous carriers. PMID: 27593409
    10. JAK3 up-regulates SGLT1 activity by increasing the carrier protein abundance in the cell membrane, an effect enforcing cellular glucose uptake into activated lymphocytes and thus contributing to the immune response. PMID: 27595398
    11. JAK3 and MCL-1 were down-regulated in patient CD8(+) T cells versus their normal counterparts, likely due to defective suppressor activity of miR-29b and miR-198 in RCC CD8(+) T cells. PMID: 27063186
    12. N225K and A550V PTPN6 mutations cause loss-of-function leading to JAK3 mediated deregulation of STAT3 pathway and uncover a mechanism that tumor cells can use to control PTPN6 substrate specificity. PMID: 26565811
    13. Study shows JAK3 mutations in 16% of patients with T-cell prolymphocytic leukemia which might be an important prognostic marker. PMID: 26493028
    14. This study showed that JAK3 is a powerful negative regulator of the creatine transporter SLC6A8. PMID: 26666525
    15. Letter/Case Report: ersistent rotavirus diarrhea post-transplant in a novel JAK3-SCID patient after vaccination. PMID: 26248889
    16. interleukin-4 regulates hematopoietic lineage choice by activating the JAK3-STAT6 pathway, which causes dendritic-cell-specific DNA demethylation. PMID: 26829670
    17. Foxp3 has a rapid turn over in Treg partly controlled at the transcriptional level by the JAK/STAT pathway PMID: 27077371
    18. Results confirm that JAK3 is mutated in T-PLL and underscore the potential therapeutical relevance of epigenetic regulator. PMID: 26917488
    19. Data provide the first evidence that de-regulated Jak3/STAT3/STAT5 signalling in CTCL cells represses the expression of the gene encoding miR-22, a novel tumor suppressor miRNA. PMID: 26244872
    20. Study describes three patients with a novel deep intronic mis-splicing mutation in JAK3 as a cause of T-B+NK- severe combined immunodeficiency. PMID: 26769277
    21. Jak3 has a role in promoting mucosal tolerance through suppressed expression and limiting activation of TLRs thereby preventing intestinal and systemic chronic low-grade inflammation and associated obesity and MetS PMID: 26451047
    22. that JAK3 may contribute to the pathogenesis of pediatric ALL and serve as an important therapeutic target which can be leveraged to improve outcomes for pediatric patients with ALL. PMID: 25146434
    23. These results do not confirm association between JAK3 polymorphisms and cardiovascular disease in rheumatoid arthritis. PMID: 25815310
    24. Activating Janus kinase 3 mutation is associated with mycosis fungoides. PMID: 26082451
    25. The study reports the first patient with relapsed pediatric Early T-cell precursor acute lymphoblastic leukemia to exhibit a homozygous JAK3 activating mutation, V674A, caused by acquired uniparental disomy. PMID: 25430085
    26. JAK inhibitor ruxolitinib (that inhibits mainly the JAK3/STAT5 pathway) affects key characteristics of human NK cells, such as cytokine-induced expansion and killing via an impaired cytokine-mediated NK cell activation PMID: 25832652
    27. Case Report: JAK3 mutations giving rise to common variable immunodeficiency mimicking late-onset combined immunodeficiency. PMID: 26182690
    28. These results suggest irreversible inhibitors of this class may be useful selective agents, both as tools to probe Jak3 biology and potentially as therapies for autoimmune diseases. PMID: 25552479
    29. no JAK3 mutations (A572V or A573V) were identified in samples of NK cell neoplasms and EBV-associated T/NK cell lymphoproliferative diseasein Japan PMID: 23808814
    30. in extranodal natural killer/T cell lymphomas, phosphorylated JAK3 expression does not necessarily harbor exon 13 mutations. PMID: 24965108
    31. JAK3/STAT5 signaling via IL-2 receptor is necessary to maintain the long-term expression of the high-affinity alpha beta gamma(c)-receptor of IL-2 and optimal proliferation of blood lymphocytes. PMID: 25509109
    32. Report activated JAK3 variant in myelodysplastic syndromes. PMID: 24674452
    33. we not only characterize Jak3 interaction with Shc, but also demonstrate the molecular mechanism of intracellular regulation of Jak3 activation PMID: 24795043
    34. The finding of recurrent activating JAK3 mutations in patients with T-PLL could enable the use of JAK3 inhibitors to treat patients with this unfavorable malignancy who otherwise have a very poor prognosis. PMID: 24446122
    35. JAK3 is a powerful regulator of the peptide transporters PEPT1 and PEPT2. PMID: 23934551
    36. In cervical cancer cells the phosphorylation of STAT5/JAK3 increases at low doses of IL-2 but significantly decreases at high doses. PMID: 24548303
    37. JAK3 mutations are found in a subset of clear cell renal cell carcinoma patients and may be associated with its development and a greater risk of metastases PMID: 21868263
    38. the essential role of Jak3 in the colon where it facilitated mucosal differentiation by promoting the expression of differentiation markers and enhanced colonic barrier functions through AJ localization of beta-catenin. PMID: 24045942
    39. Mutations of SETBP1 and JAK3 were common recurrent secondary events presumed to be involved in tumor progression and were associated with poor clinical outcomes. PMID: 23832011
    40. Hypomorphic Janus kinase 3 mutations result in a spectrum of immune defects, including partial maternal T-cell engraftment. PMID: 23384681
    41. analysis of the JAK-Fes-phospholipase D signaling pathway that is enhanced in highly proliferative breast cancer cells PMID: 23404507
    42. Data suggest that cross-talk occurs between cAMP/PKA and the IL-2R beta/Jak3/Stat5b cascade in T-cells. PMID: 23341462
    43. Data indicate that the invasive phenotype of MDA-MB-231 cells is mediated by phospholipase D2 (PLD2) under direct regulation of both Janus kinase 3 (JAK3) and tepidermal growth factor receptor (EGFR). PMID: 23238254
    44. analysis of molecular mechanism of interactions between Jak3 and cytoskeletal proteins where tyrosine phosphorylation of the SH2 domain acted as an intramolecular switch for the interactions between Jak3 and cytoskeletal proteins PMID: 23012362
    45. Data suggest that Janus kinase 3 (JAK3) may play a significant role in the pathogenesis of natural killer/T-cell lymphoma (NKTCL). PMID: 22705984
    46. JAK3 mutations are associated with response to therapy in T-cell acute lymphoblastic leukemia. PMID: 22425895
    47. peripheral blood lymphocytes (PBLs) of ovarian cancer patients showed lower JAK3, CD3-zeta molecules expression levels, as well as lower STAT3 and CD3-zeta phosphorylation levels than cells of control. PMID: 22221142
    48. analysis of the biochemistry, immunological functions, and clinical significance of JAK3 [review] PMID: 22130498
    49. Data imply that IL-21-mediated signaling is critical for long-lived humoral immunity and to restore antibody responses in IL2RG/JAK3-deficient patients after hematopoietic cell transplantation. PMID: 22039266
    50. Review: discuss role of JAK3 inhibitors as immunosuppressive agents in kidney transplantation. PMID: 21971513

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  • 相关疾病：
    Severe combined immunodeficiency autosomal recessive T-cell-negative/B-cell-positive/NK-cell-negative (T(-)B(+)NK(-) SCID)
  • 亚细胞定位：
    Endomembrane system; Peripheral membrane protein. Cytoplasm.
  • 蛋白家族：
    Protein kinase superfamily, Tyr protein kinase family, JAK subfamily
  • 组织特异性：
    In NK cells and an NK-like cell line but not in resting T-cells or in other tissues. The S-form is more commonly seen in hematopoietic lines, whereas the B-form is detected in cells both of hematopoietic and epithelial origins.
  • 数据库链接：

    HGNC: 6193

    OMIM: 600173

    KEGG: hsa:3718

    STRING: 9606.ENSP00000391676

    UniGene: Hs.515247