B Lymphocyte Receptor

14,000,000 Leading Edge Experts on the ideXlab platform

Scan Science and Technology

Contact Leading Edge Experts & Companies

Scan Science and Technology

Contact Leading Edge Experts & Companies

The Experts below are selected from a list of 14337 Experts worldwide ranked by ideXlab platform

Morton J Cowan - One of the best experts on this subject based on the ideXlab platform.

  • A novel missense RAG-1 mutation results in T^−B^−NK^+ SCID in AthaBascan-speaking Dine Indians from the Canadian Northwest Territories
    European Journal of Human Genetics, 2009
    Co-Authors: Zheng Xiao, Steven M Yannone, Elizabeth Dunn, Morton J Cowan
    Abstract:

    DNA douBle-strand repair factors in the non-homologous end joining (NHEJ) pathway resolve DNA douBle-strand Breaks introduced By the recomBination-activating gene (RAG) proteins during V(D)J recomBination of T and B Lymphocyte Receptor genes. Defective NHEJ and suBsequent failure of V(D)J recomBination leads to severe comBined immunodeficiency disease (SCID). We originally linked T^−B^−NK^+ SCID in AthaBascan-speaking Native Americans in the Southwestern US and Northwest Territories of Canada to chromosome 10. However, despite a common ancestry, the null mutation in the Artemis gene that we found to Be causal in the SCID among the Navajo and Apache Indians was not present in the Dine Indians in the Northwest Territories. We now report a novel homozygous missense mutation (R776W) in RAG-1 in three children with T^−B^−NK^+ SCID from two related families of AthaBascan-speaking Dine Indians in the Canadian Northwest Territories. As expected, we found no increased sensitivity to ionizing radiation in patient fiBroBlasts. The impaired activity of this RAG-1 mutant in V(D)J recomBination was confirmed By the EGFP-Based V(D)J recomBination assays. Overexpression of wild type RAG-1 in patient fiBroBlasts complemented V(D)J recomBination, with recovery of Both coding and signal joint formation. Our results indicate that the novel R776W missense mutation in RAG-1 is causal in the T^−B^−NK^+ SCID phenotype in AthaBascan-speaking Dine Indians from the Canadian Northwest Territories.

  • A novel missense RAG-1 mutation results in T − B − NK + SCID in AthaBascan-speaking Dine Indians from the Canadian Northwest Territories
    European journal of human genetics : EJHG, 2008
    Co-Authors: Zheng Xiao, Steven M Yannone, Elizabeth Dunn, Morton J Cowan
    Abstract:

    DNA douBle-strand repair factors in the non-homologous end joining (NHEJ) pathway resolve DNA douBle-strand Breaks introduced By the recomBination-activating gene (RAG) proteins during V(D)J recomBination of T and B Lymphocyte Receptor genes. Defective NHEJ and suBsequent failure of V(D)J recomBination leads to severe comBined immunodeficiency disease (SCID). We originally linked T−B−NK+ SCID in AthaBascan-speaking Native Americans in the Southwestern US and Northwest Territories of Canada to chromosome 10. However, despite a common ancestry, the null mutation in the Artemis gene that we found to Be causal in the SCID among the Navajo and Apache Indians was not present in the Dine Indians in the Northwest Territories. We now report a novel homozygous missense mutation (R776W) in RAG-1 in three children with T−B−NK+ SCID from two related families of AthaBascan-speaking Dine Indians in the Canadian Northwest Territories. As expected, we found no increased sensitivity to ionizing radiation in patient fiBroBlasts. The impaired activity of this RAG-1 mutant in V(D)J recomBination was confirmed By the EGFP-Based V(D)J recomBination assays. Overexpression of wild type RAG-1 in patient fiBroBlasts complemented V(D)J recomBination, with recovery of Both coding and signal joint formation. Our results indicate that the novel R776W missense mutation in RAG-1 is causal in the T−B−NK+ SCID phenotype in AthaBascan-speaking Dine Indians from the Canadian Northwest Territories.

Li Zhuo - One of the best experts on this subject based on the ideXlab platform.

  • high throughput sequencing analysis of genes encoding the B Lymphocyte Receptor heavy chain cdr3 in renal and peripheral Blood of iga nephropathy
    Bioscience Reports, 2019
    Co-Authors: Dapeng Chen, Zheng Zhang, Yue Yang, Quan Hong, Li Zhuo
    Abstract:

    AIM IgA nephropathy (IgAN) is one of the most common chronic glomerulonephritis. Its etiology and pathogenesis remain unclear. We thus explored the immune repertoire of the B-cell Receptor (BCR) and the heavy-chain complementarity-determining region 3 (CDR3) in renal tissue and peripheral Blood of IgAN patients. METHOD Total RNAs extracted from renal tissues and peripheral Blood of patients and peripheral Blood of healthy controls (HCs) were analyzed via high-throughput multiplex PCR sequencing. We amplified and sequenced BCR heavy-chain CDR3 regions to explore repertoire diversity, V/J gene family distriBution, CDR3 lengths, BCR heavy-chain variants, consistency Between tissue and peripheral Blood data, and clones 'shared' By these Bodily compartments. RESULTS We identified the renal tissue and peripheral Blood BCR heavy-chain CDR3 immune repertoires of 15 IgAN patients. Top1 could Be more readily cloned from peripheral Blood of patients than from controls (P<0.05), the average CDR3 length was significantly shorter in patients than in HCs (P<0.05), the variant frequency of the gene encoding the BCR heavy chain was higher in patients than in HCs (P<0.05), and the BCR variant frequency was highest in IgAN kidney tissue. Preliminary screening for 'shared' clones showed that, in at least 13 patients, the 'ALYFHNSAY', 'ARWGPMYYYMDV', 'ARDQGALNA', and 'ARVDNPADF' CDR3 sequences were evident in peripheral Blood samples from patients, But not HCs. CONCLUSIONS We found that the 'ALYFHNSAY', 'ARWGPMYYYMDV', 'ARDQGALNA', and 'ARVDNPADF' clonal sequences may Be useful for noninvasive diagnosis and treatment planning in IgAN.

  • High-throughput sequencing analysis of genes encoding the B-Lymphocyte Receptor heavy-chain CDR3 in renal and peripheral Blood of IgA nephropathy
    Bioscience reports, 2019
    Co-Authors: Dapeng Chen, Zheng Zhang, Yue Yang, Quan Hong, Li Zhuo
    Abstract:

    AIM IgA nephropathy (IgAN) is one of the most common chronic glomerulonephritis. Its etiology and pathogenesis remain unclear. We thus explored the immune repertoire of the B-cell Receptor (BCR) and the heavy-chain complementarity-determining region 3 (CDR3) in renal tissue and peripheral Blood of IgAN patients. METHOD Total RNAs extracted from renal tissues and peripheral Blood of patients and peripheral Blood of healthy controls (HCs) were analyzed via high-throughput multiplex PCR sequencing. We amplified and sequenced BCR heavy-chain CDR3 regions to explore repertoire diversity, V/J gene family distriBution, CDR3 lengths, BCR heavy-chain variants, consistency Between tissue and peripheral Blood data, and clones 'shared' By these Bodily compartments. RESULTS We identified the renal tissue and peripheral Blood BCR heavy-chain CDR3 immune repertoires of 15 IgAN patients. Top1 could Be more readily cloned from peripheral Blood of patients than from controls (P

Zheng Xiao - One of the best experts on this subject based on the ideXlab platform.

  • A novel missense RAG-1 mutation results in T^−B^−NK^+ SCID in AthaBascan-speaking Dine Indians from the Canadian Northwest Territories
    European Journal of Human Genetics, 2009
    Co-Authors: Zheng Xiao, Steven M Yannone, Elizabeth Dunn, Morton J Cowan
    Abstract:

    DNA douBle-strand repair factors in the non-homologous end joining (NHEJ) pathway resolve DNA douBle-strand Breaks introduced By the recomBination-activating gene (RAG) proteins during V(D)J recomBination of T and B Lymphocyte Receptor genes. Defective NHEJ and suBsequent failure of V(D)J recomBination leads to severe comBined immunodeficiency disease (SCID). We originally linked T^−B^−NK^+ SCID in AthaBascan-speaking Native Americans in the Southwestern US and Northwest Territories of Canada to chromosome 10. However, despite a common ancestry, the null mutation in the Artemis gene that we found to Be causal in the SCID among the Navajo and Apache Indians was not present in the Dine Indians in the Northwest Territories. We now report a novel homozygous missense mutation (R776W) in RAG-1 in three children with T^−B^−NK^+ SCID from two related families of AthaBascan-speaking Dine Indians in the Canadian Northwest Territories. As expected, we found no increased sensitivity to ionizing radiation in patient fiBroBlasts. The impaired activity of this RAG-1 mutant in V(D)J recomBination was confirmed By the EGFP-Based V(D)J recomBination assays. Overexpression of wild type RAG-1 in patient fiBroBlasts complemented V(D)J recomBination, with recovery of Both coding and signal joint formation. Our results indicate that the novel R776W missense mutation in RAG-1 is causal in the T^−B^−NK^+ SCID phenotype in AthaBascan-speaking Dine Indians from the Canadian Northwest Territories.

  • A novel missense RAG-1 mutation results in T − B − NK + SCID in AthaBascan-speaking Dine Indians from the Canadian Northwest Territories
    European journal of human genetics : EJHG, 2008
    Co-Authors: Zheng Xiao, Steven M Yannone, Elizabeth Dunn, Morton J Cowan
    Abstract:

    DNA douBle-strand repair factors in the non-homologous end joining (NHEJ) pathway resolve DNA douBle-strand Breaks introduced By the recomBination-activating gene (RAG) proteins during V(D)J recomBination of T and B Lymphocyte Receptor genes. Defective NHEJ and suBsequent failure of V(D)J recomBination leads to severe comBined immunodeficiency disease (SCID). We originally linked T−B−NK+ SCID in AthaBascan-speaking Native Americans in the Southwestern US and Northwest Territories of Canada to chromosome 10. However, despite a common ancestry, the null mutation in the Artemis gene that we found to Be causal in the SCID among the Navajo and Apache Indians was not present in the Dine Indians in the Northwest Territories. We now report a novel homozygous missense mutation (R776W) in RAG-1 in three children with T−B−NK+ SCID from two related families of AthaBascan-speaking Dine Indians in the Canadian Northwest Territories. As expected, we found no increased sensitivity to ionizing radiation in patient fiBroBlasts. The impaired activity of this RAG-1 mutant in V(D)J recomBination was confirmed By the EGFP-Based V(D)J recomBination assays. Overexpression of wild type RAG-1 in patient fiBroBlasts complemented V(D)J recomBination, with recovery of Both coding and signal joint formation. Our results indicate that the novel R776W missense mutation in RAG-1 is causal in the T−B−NK+ SCID phenotype in AthaBascan-speaking Dine Indians from the Canadian Northwest Territories.

Dapeng Chen - One of the best experts on this subject based on the ideXlab platform.

  • high throughput sequencing analysis of genes encoding the B Lymphocyte Receptor heavy chain cdr3 in renal and peripheral Blood of iga nephropathy
    Bioscience Reports, 2019
    Co-Authors: Dapeng Chen, Zheng Zhang, Yue Yang, Quan Hong, Li Zhuo
    Abstract:

    AIM IgA nephropathy (IgAN) is one of the most common chronic glomerulonephritis. Its etiology and pathogenesis remain unclear. We thus explored the immune repertoire of the B-cell Receptor (BCR) and the heavy-chain complementarity-determining region 3 (CDR3) in renal tissue and peripheral Blood of IgAN patients. METHOD Total RNAs extracted from renal tissues and peripheral Blood of patients and peripheral Blood of healthy controls (HCs) were analyzed via high-throughput multiplex PCR sequencing. We amplified and sequenced BCR heavy-chain CDR3 regions to explore repertoire diversity, V/J gene family distriBution, CDR3 lengths, BCR heavy-chain variants, consistency Between tissue and peripheral Blood data, and clones 'shared' By these Bodily compartments. RESULTS We identified the renal tissue and peripheral Blood BCR heavy-chain CDR3 immune repertoires of 15 IgAN patients. Top1 could Be more readily cloned from peripheral Blood of patients than from controls (P<0.05), the average CDR3 length was significantly shorter in patients than in HCs (P<0.05), the variant frequency of the gene encoding the BCR heavy chain was higher in patients than in HCs (P<0.05), and the BCR variant frequency was highest in IgAN kidney tissue. Preliminary screening for 'shared' clones showed that, in at least 13 patients, the 'ALYFHNSAY', 'ARWGPMYYYMDV', 'ARDQGALNA', and 'ARVDNPADF' CDR3 sequences were evident in peripheral Blood samples from patients, But not HCs. CONCLUSIONS We found that the 'ALYFHNSAY', 'ARWGPMYYYMDV', 'ARDQGALNA', and 'ARVDNPADF' clonal sequences may Be useful for noninvasive diagnosis and treatment planning in IgAN.

  • High-throughput sequencing analysis of genes encoding the B-Lymphocyte Receptor heavy-chain CDR3 in renal and peripheral Blood of IgA nephropathy
    Bioscience reports, 2019
    Co-Authors: Dapeng Chen, Zheng Zhang, Yue Yang, Quan Hong, Li Zhuo
    Abstract:

    AIM IgA nephropathy (IgAN) is one of the most common chronic glomerulonephritis. Its etiology and pathogenesis remain unclear. We thus explored the immune repertoire of the B-cell Receptor (BCR) and the heavy-chain complementarity-determining region 3 (CDR3) in renal tissue and peripheral Blood of IgAN patients. METHOD Total RNAs extracted from renal tissues and peripheral Blood of patients and peripheral Blood of healthy controls (HCs) were analyzed via high-throughput multiplex PCR sequencing. We amplified and sequenced BCR heavy-chain CDR3 regions to explore repertoire diversity, V/J gene family distriBution, CDR3 lengths, BCR heavy-chain variants, consistency Between tissue and peripheral Blood data, and clones 'shared' By these Bodily compartments. RESULTS We identified the renal tissue and peripheral Blood BCR heavy-chain CDR3 immune repertoires of 15 IgAN patients. Top1 could Be more readily cloned from peripheral Blood of patients than from controls (P

Michael P Cooke - One of the best experts on this subject based on the ideXlab platform.

  • Production of Ins(1,3,4,5)P_4 mediated By the kinase ItpkB inhiBits store-operated calcium channels and regulates B cell selection and activation
    Nature Immunology, 2007
    Co-Authors: Andrew T Miller, Mark Sandberg, Karsten Sauer, Michael Young, Yina H. Huang, Susan Sutton, Michael P Cooke
    Abstract:

    Antigen Receptor–mediated production of inositol-1,4,5-trisphosphate (Ins(1,4,5)P_3) in Lymphocytes triggers the release of Ca^2+ from intracellular stores; this release of Ca^2+ results in the opening of store-operated Ca^2+ channels in the plasma memBrane. Here we report that mice lacking Ins(1,4,5)P_3 3-kinase B (ItpkB), which converts Ins(1,4,5)P_3 to inositol-1,3,4,5-tetrakisphosphate (Ins(1,3,4,5)P_4), had impaired B Lymphocyte development and defective immunogloBulin G3 antiBody responses to a T Lymphocyte–independent antigen. ItpkB-deficient B Lymphocytes had the phenotypic and functional features of tolerant B Lymphocytes and showed enhanced activity of store-operated Ca^2+ channels after B Lymphocyte Receptor stimulation, which was reversed By the provision of exogenous Ins(1,3,4,5)P_4. Our data identify ItpkB and its product Ins(1,3,4,5)P_4 as inhiBitors of store-operated Ca^2+ channels and crucial regulators of B cell selection and activation.

  • Production of Ins(1,3,4,5)P4 mediated By the kinase ItpkB inhiBits store-operated calcium channels and regulates B cell selection and activation
    Nature Immunology, 2007
    Co-Authors: Andrew T Miller, Karsten Sauer, Mark L. Sandberg, Susan E. Sutton, Yina H. Huang, Mike Young, Michael P Cooke
    Abstract:

    Antigen Receptor–mediated production of inositol-1,4,5-trisphosphate (Ins(1,4,5)P3) in Lymphocytes triggers the release of Ca2+ from intracellular stores; this release of Ca2+ results in the opening of store-operated Ca2+ channels in the plasma memBrane. Here we report that mice lacking Ins(1,4,5)P3 3-kinase B (ItpkB), which converts Ins(1,4,5)P3 to inositol-1,3,4,5-tetrakisphosphate (Ins(1,3,4,5)P4), had impaired B Lymphocyte development and defective immunogloBulin G3 antiBody responses to a T Lymphocyte–independent antigen. ItpkB-deficient B Lymphocytes had the phenotypic and functional features of tolerant B Lymphocytes and showed enhanced activity of store-operated Ca2+ channels after B Lymphocyte Receptor stimulation, which was reversed By the provision of exogenous Ins(1,3,4,5)P4. Our data identify ItpkB and its product Ins(1,3,4,5)P4 as inhiBitors of store-operated Ca2+ channels and crucial regulators of B cell selection and activation.