| 别名 | Tyrosine-protein kinase JAK3, Janus kinase 3, Leukocyte janus kinase, JAK-3, L-JAK, JAK3 |
| 基因名称 | JAK3 |
| 蛋白名称 | Tyrosine-protein kinase JAK3 |
| 推荐应用 | |
| 反应物种 | Human |
| 存储缓冲液 | PBS, 50% glycerol, 0.05% Proclin 300, 0.05%BSA |
| 特异性 | Endogenous |
| 稀释度 | WB 1:500-1:2000;IHC-P 1:200-1:1000 |
| 参考分子量 | MW(Observed):125kD |
| 预测分子量 | MW(Calculated):125kD |
| 运输及保存条件 | -15°C to -25°C/1 year(Do not lower than -25°C) |
| 宿主 | Rabbit |
| 同种型 | IgG,Kappa |
| 背景介绍 | The protein encoded by this gene is a member of the Janus kinase (JAK) family of tyrosine kinases involved in cytokine receptor-mediated intracellular signal transduction. It is predominantly expressed in immune cells and transduces a signal in response to its activation via tyrosine phosphorylation by interleukin receptors. Mutations in this gene are associated with autosomal SCID (severe combined immunodeficiency disease) . |
| 细胞定位 | Cytoplasm |
| 功能 | Catalytic activity:ATP + a [protein]-L-tyrosine = ADP + a [protein]-L-tyrosine phosphate. ,Disease:Defects in JAK3 are a cause of severe combined immunodeficiency autosomal recessive T-cell-negative/B-cell-positive/NK-cell-negative (T (-) B (+) NK (-) SCID) [MIM:600802]. SCID refers to 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 with SCID present in infancy with 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. ,Domain:Possesses two phosphotransferase domains. The second one probably contains the catalytic domain (By similarity) , while the presence of slight differences suggest a different role for domain 1. ,Function:Tyrosine kinase of the non-receptor type , involved in the interleukin-2 and interleukin-4 signaling pathway. Phosphorylates STAT6 , IRS1 , IRS2 and PI3K. ,online information:JAK3 mutation db ,PTM:Tyrosine phosphorylated in response to IL-2 and IL-4. ,similarity:Belongs to the protein kinase superfamily. Tyr protein kinase family. JAK subfamily. ,similarity:Contains 1 FERM domain. ,similarity:Contains 1 protein kinase domain. |
| 纯化 | Protein A |
| Clonality | Monoclonal |
| 修饰类型 | Unmodified |
| 细胞定位 | Cytoplasm |
| 组织表达 | Spleen, Lymph node, Thymus, T-cell lymphoma, Acute lymphoblastic leukemia, Breast carcinoma |
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