其他名称： BLNK; BASH; SLP65; B-cell linker protein; B-cell adapter containing a SH2 domain protein; B-cell adapter containing a Src homology 2 domain protein; Cytoplasmic adapter protein; Src homology 2 domain-containing leukocyte protein of 65 kDa;
信号通路： B_Cell_Antigen;Primary immunodeficiency;
功能： disease:Defects in BLNK are the cause of hypoglobulinemia and absent B-cells [MIM:604515]. This is a developmental blockage at the pro- to pre-B-cell transition.,disease:In 6 of 34 childhood pre-B acute lymphoblastic leukemia (ALL) samples that were tested showed a complete loss or drastic reduction of BLNK expression.,function:Functions as a central linker protein that bridges kinases associated with the B-cell receptor (BCR) with a multitude of signaling pathways, regulating biological outcomes of B-cell function and development. Plays a role in the activation of ERK/EPHB2, MAP kinase p38 and JNK. Modulates AP1 activation. Important for the activation of NF-kappa-B and NFAT. Plays an important role in BCR-mediated PLCG1 and PLCG2 activation and Ca(2+) mobilization and is required for trafficking of the BCR to late endosomes. However, does not seem to be required for pre-BCR-mediated activation of MAP kinase and phosphatidyl-inositol 3 (PI3) kinase signaling. May be required for the RAC1-JNK pathway. Plays a critical role in orchestrating the pro-B cell to pre-B cell transition (By similarity). Plays an important role in BCR-induced B-cell apoptosis.,online information:BLNK mutation db,PTM:Following BCR activation, phosphorylated on tyrosine residues by SYK and LYN. When phosphorylated, serves as a scaffold to assemble downstream targets of antigen activation, including PLCG1, VAV1, GRB2 and NCK1. Phosphorylation of Tyr-84, Tyr-178 and Tyr-189 facilitates PLCG1 binding. Phosphorylation of Tyr-96 facilitates BTK binding. Phosphorylation of Tyr-72 facilitates VAV1 and NCK1 binding. Phosphorylation is required for both Ca(2+) and MAPK signaling pathways.,similarity:Contains 1 SH2 domain.,subcellular location:BCR activation results in the translocation to membrane fraction.,subunit:Associates with PLCG1, VAV1 and NCK1 in a B-cell antigen receptor-dependent fashion. Interacts with VAV3, PLCG2 and GRB2. Interacts through its SH2 domain with CD79A.,tissue specificity:Expressed in B-cell lineage and fibroblast cell lines (at protein level). Highest levels of expression in the spleen, with lower levels in the liver, kidney, pancreas, small intestines and colon.,
相关产品： RS0001,RS0002,YM3028,YM3029
细胞定位： Cytoplasm . Cell membrane . BCR activation results in the translocation to membrane fraction.
组织表达： Expressed in B-cell lineage and fibroblast cell lines (at protein level). Highest levels of expression in the spleen, with lower levels in the liver, kidney, pancreas, small intestines and colon.
其他名称： BLNK; BASH; SLP65; B-cell linker protein; B-cell adapter containing a SH2 domain protein; B-cell adapter containing a Src homology 2 domain protein; Cytoplasmic adapter protein; Src homology 2 domain-containing leukocyte protein of 65 kDa;
信号通路： B_Cell_Antigen;Primary immunodeficiency;
功能： disease:Defects in BLNK are the cause of hypoglobulinemia and absent B-cells [MIM:604515]. This is a developmental blockage at the pro- to pre-B-cell transition.,disease:In 6 of 34 childhood pre-B acute lymphoblastic leukemia (ALL) samples that were tested showed a complete loss or drastic reduction of BLNK expression.,function:Functions as a central linker protein that bridges kinases associated with the B-cell receptor (BCR) with a multitude of signaling pathways, regulating biological outcomes of B-cell function and development. Plays a role in the activation of ERK/EPHB2, MAP kinase p38 and JNK. Modulates AP1 activation. Important for the activation of NF-kappa-B and NFAT. Plays an important role in BCR-mediated PLCG1 and PLCG2 activation and Ca(2+) mobilization and is required for trafficking of the BCR to late endosomes. However, does not seem to be required for pre-BCR-mediated activation of MAP kinase and phosphatidyl-inositol 3 (PI3) kinase signaling. May be required for the RAC1-JNK pathway. Plays a critical role in orchestrating the pro-B cell to pre-B cell transition (By similarity). Plays an important role in BCR-induced B-cell apoptosis.,online information:BLNK mutation db,PTM:Following BCR activation, phosphorylated on tyrosine residues by SYK and LYN. When phosphorylated, serves as a scaffold to assemble downstream targets of antigen activation, including PLCG1, VAV1, GRB2 and NCK1. Phosphorylation of Tyr-84, Tyr-178 and Tyr-189 facilitates PLCG1 binding. Phosphorylation of Tyr-96 facilitates BTK binding. Phosphorylation of Tyr-72 facilitates VAV1 and NCK1 binding. Phosphorylation is required for both Ca(2+) and MAPK signaling pathways.,similarity:Contains 1 SH2 domain.,subcellular location:BCR activation results in the translocation to membrane fraction.,subunit:Associates with PLCG1, VAV1 and NCK1 in a B-cell antigen receptor-dependent fashion. Interacts with VAV3, PLCG2 and GRB2. Interacts through its SH2 domain with CD79A.,tissue specificity:Expressed in B-cell lineage and fibroblast cell lines (at protein level). Highest levels of expression in the spleen, with lower levels in the liver, kidney, pancreas, small intestines and colon.,
相关产品： RS0001,RS0002,YM3028,YM3029
细胞定位： Cytoplasm . Cell membrane . BCR activation results in the translocation to membrane fraction.
组织表达： Expressed in B-cell lineage and fibroblast cell lines (at protein level). Highest levels of expression in the spleen, with lower levels in the liver, kidney, pancreas, small intestines and colon.
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