Description
IL-2-inducible T cell kinase is a member of the protein kinase superfamily, Tyr protein kinase family, and TEC subfamily. It contains 1 Btk-type zinc finger, 1 PH domain, 1 protein kinase domain, 1 SH2 domain, and 1 SH3 domain. As an intracellular kinase expressed in T-cells, IL-2-inducible T cell kinase contains both SH2 and SH3 domains which are often found in intracellular kinases. It is thought to play a role in T-cell proliferation and differentiation. It regulates the development, function, and differentiation of conventional T-cells and nonconventional NKT-cells. IL-2-inducible T cell kinase also plays an essential role in the regulation of the adaptive immune response. Effects in IL-2-inducible T cell kinase are the cause of lymphoproliferative syndrome EBV-associated autosomal type 1 (LPSA1). LPSA1 is a rare immunodeficiency characterized by extreme susceptibility to infection with Epstein-Barr virus (EBV). Inadequate immune response to EBV can have a fatal outcome. Clinical features include splenomegaly, lymphadenopathy, anemia, thrombocytopenia, pancytopenia, recurrent infections. There is an increased risk of lymphoma.
Form
Lyophilized from sterile 50 mM Tris, 150 mM NaCl, pH 7.5, 25 % glycerol, 0.5 mM PMSF, 0.5 mM TCEP, 0.1 mM EDTA, 0.5 mM GSH, 5 % trehalose, 5% mannitol and 0.01% Tween80.
