免疫原
Purified recombinant fragment of human JAK3 expressed in E. Coli.
特异性
JAK3 Monoclonal Antibody detects endogenous levels of JAK3 protein.
组成(Formulation)
Ascitic fluid containing 0.03% sodium azide,0.5% BSA, 50%glycerol.
稀释比例
Western Blot: 1/500 - 1/2000. Immunofluorescence: 1/200 - 1/1000. Flow cytometry: 1/200 - 1/400. ELISA: 1/10000. Not yet tested in other applications.
纯化工艺(Immunogen)
Affinity purification
背景(Background)
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). [provided by RefSeq, Jul 2008],
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
蛋白名称
Tyrosine-protein kinase JAK3
其他名称
JAK3; Tyrosine-protein kinase JAK3; Janus kinase 3; JAK-3; Leukocyte janus kinase; L-JAK
Fields
>>Chemokine signaling pathway;>>PI3K-Akt signaling pathway;>>Necroptosis;>>Signaling pathways regulating pluripotency of stem cells;>>JAK-STAT signaling pathway;>>Th1 and Th2 cell differentiation;>>Th17 cell differentiation;>>Hepatitis B;>>Measles;>>Human T-cell leukemia virus 1 infection;>>Epstein-Barr virus infection;>>Pathways in cancer;>>Viral carcinogenesis;>>Non-small cell lung cancer;>>Primary immunodeficiency
细胞定位
Endomembrane system ; Peripheral membrane protein . Cytoplasm .
组织表达
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.