Immunological phenotype of the murine Lrba knockout
Biallelic mutations in the human lipopolysaccharide responsive beige‐like anchor (LRBA) gene lead to a primary immunodeficiency known as LRBA deficiency, characterized by a broad range of clinical manifestations including autoimmunity, organomegaly, hypogammaglobulinemia and recurrent infections. Co...
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| Published in | Immunology and cell biology Vol. 95; no. 9; pp. 789 - 802 |
|---|---|
| Main Authors | , , , , , , , , , , , , |
| Format | Journal Article |
| Language | English |
| Published |
England
Nature Publishing Group
01.10.2017
Blackwell Science Ltd |
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| Online Access | Get full text |
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| Abstract | Biallelic mutations in the human lipopolysaccharide responsive beige‐like anchor (LRBA) gene lead to a primary immunodeficiency known as LRBA deficiency, characterized by a broad range of clinical manifestations including autoimmunity, organomegaly, hypogammaglobulinemia and recurrent infections. Considering the phenotypic heterogeneity in patients and the severity of the disease, our aim was to assess the role of LRBA in immune cells and to understand the underlying pathomechanisms through the study of a Lrba knockout (Lrba−/−) mouse model. LRBA‐deficient mice did not show severe clinical or immunological signs of disease, either at steady state under specific‐pathogen‐free conditions, after vaccination with T‐dependent and T‐independent antigens, or in the context of acute infections with lymphocytic choriomeningitis virus (LCMV) or Salmonella Typhimurium. Although Lrba−/− mice were able to produce normal serum immunoglobulin M (IgM) and IgG and to mount a specific immune response after immunization, they showed elevated serum and secretory basal IgA levels. LRBA was dispensable for B‐ and T‐cell development, as well as for in vitro B‐cell proliferation, survival, isotype switching and plasmablast differentiation. Interestingly, Lrba−/− mice displayed decreased cytotoxic T‐lymphocyte‐associated protein‐4 (CTLA‐4) expression by regulatory T cells and activated conventional CD4+ and CD8+ T lymphocytes, reduced frequency of peritoneal B‐1a cells along with diminished interleukin‐10 production and increased percentages of T follicular helper cells in Peyer's patches, but without developing overt signs of autoimmunity. Our findings expand the role of LRBA in immune regulatory mechanisms previously reported in patients, and suggest a novel role in IgA production that is crucial for the protection of mucosal surfaces and gut‐associated immune tolerance. |
|---|---|
| AbstractList | Biallelic mutations in the human
lipopolysaccharide responsive beige‐like anchor
(
LRBA
) gene lead to a primary immunodeficiency known as LRBA deficiency, characterized by a broad range of clinical manifestations including autoimmunity, organomegaly, hypogammaglobulinemia and recurrent infections. Considering the phenotypic heterogeneity in patients and the severity of the disease, our aim was to assess the role of LRBA in immune cells and to understand the underlying pathomechanisms through the study of a
Lrba
knockout (
Lrba
−/−
) mouse model. LRBA‐deficient mice did not show severe clinical or immunological signs of disease, either at steady state under specific‐pathogen‐free conditions, after vaccination with T‐dependent and T‐independent antigens, or in the context of acute infections with lymphocytic choriomeningitis virus (LCMV) or
Salmonella Typhimurium
. Although
Lrba
−/−
mice were able to produce normal serum immunoglobulin M (IgM) and IgG and to mount a specific immune response after immunization, they showed elevated serum and secretory basal IgA levels. LRBA was dispensable for B‐ and T‐cell development, as well as for
in vitro
B‐cell proliferation, survival, isotype switching and plasmablast differentiation. Interestingly,
Lrba
−/−
mice displayed decreased cytotoxic T‐lymphocyte‐associated protein‐4 (CTLA‐4) expression by regulatory T cells and activated conventional CD4
+
and CD8
+
T lymphocytes, reduced frequency of peritoneal B‐1a cells along with diminished interleukin‐10 production and increased percentages of T follicular helper cells in Peyer's patches, but without developing overt signs of autoimmunity. Our findings expand the role of LRBA in immune regulatory mechanisms previously reported in patients, and suggest a novel role in IgA production that is crucial for the protection of mucosal surfaces and gut‐associated immune tolerance. Biallelic mutations in the human lipopolysaccharide responsive beige-like anchor (LRBA) gene lead to a primary immunodeficiency known as LRBA deficiency, characterized by a broad range of clinical manifestations including autoimmunity, organomegaly, hypogammaglobulinemia and recurrent infections. Considering the phenotypic heterogeneity in patients and the severity of the disease, our aim was to assess the role of LRBA in immune cells and to understand the underlying pathomechanisms through the study of a Lrba knockout (Lrba-/- ) mouse model. LRBA-deficient mice did not show severe clinical or immunological signs of disease, either at steady state under specific-pathogen-free conditions, after vaccination with T-dependent and T-independent antigens, or in the context of acute infections with lymphocytic choriomeningitis virus (LCMV) or Salmonella Typhimurium. Although Lrba-/- mice were able to produce normal serum immunoglobulin M (IgM) and IgG and to mount a specific immune response after immunization, they showed elevated serum and secretory basal IgA levels. LRBA was dispensable for B- and T-cell development, as well as for in vitro B-cell proliferation, survival, isotype switching and plasmablast differentiation. Interestingly, Lrba-/- mice displayed decreased cytotoxic T-lymphocyte-associated protein-4 (CTLA-4) expression by regulatory T cells and activated conventional CD4+ and CD8+ T lymphocytes, reduced frequency of peritoneal B-1a cells along with diminished interleukin-10 production and increased percentages of T follicular helper cells in Peyer's patches, but without developing overt signs of autoimmunity. Our findings expand the role of LRBA in immune regulatory mechanisms previously reported in patients, and suggest a novel role in IgA production that is crucial for the protection of mucosal surfaces and gut-associated immune tolerance. Biallelic mutations in the human lipopolysaccharide responsive beige-like anchor (LRBA) gene lead to a primary immunodeficiency known as LRBA deficiency, characterized by a broad range of clinical manifestations including autoimmunity, organomegaly, hypogammaglobulinemia and recurrent infections. Considering the phenotypic heterogeneity in patients and the severity of the disease, our aim was to assess the role of LRBA in immune cells and to understand the underlying pathomechanisms through the study of a Lrba knockout (Lrba-/-) mouse model. LRBA-deficient mice did not show severe clinical or immunological signs of disease, either at steady state under specific-pathogen-free conditions, after vaccination with T-dependent and T-independent antigens, or in the context of acute infections with lymphocytic choriomeningitis virus (LCMV) or Salmonella Typhimurium. Although Lrba-/- mice were able to produce normal serum immunoglobulin M (IgM) and IgG and to mount a specific immune response after immunization, they showed elevated serum and secretory basal IgA levels. LRBA was dispensable for B- and T-cell development, as well as for in vitro B-cell proliferation, survival, isotype switching and plasmablast differentiation. Interestingly, Lrba-/- mice displayed decreased cytotoxic T-lymphocyte-associated protein-4 (CTLA-4) expression by regulatory T cells and activated conventional CD4+ and CD8+ T lymphocytes, reduced frequency of peritoneal B-1a cells along with diminished interleukin-10 production and increased percentages of T follicular helper cells in Peyer's patches, but without developing overt signs of autoimmunity. Our findings expand the role of LRBA in immune regulatory mechanisms previously reported in patients, and suggest a novel role in IgA production that is crucial for the protection of mucosal surfaces and gut-associated immune tolerance.Biallelic mutations in the human lipopolysaccharide responsive beige-like anchor (LRBA) gene lead to a primary immunodeficiency known as LRBA deficiency, characterized by a broad range of clinical manifestations including autoimmunity, organomegaly, hypogammaglobulinemia and recurrent infections. Considering the phenotypic heterogeneity in patients and the severity of the disease, our aim was to assess the role of LRBA in immune cells and to understand the underlying pathomechanisms through the study of a Lrba knockout (Lrba-/-) mouse model. LRBA-deficient mice did not show severe clinical or immunological signs of disease, either at steady state under specific-pathogen-free conditions, after vaccination with T-dependent and T-independent antigens, or in the context of acute infections with lymphocytic choriomeningitis virus (LCMV) or Salmonella Typhimurium. Although Lrba-/- mice were able to produce normal serum immunoglobulin M (IgM) and IgG and to mount a specific immune response after immunization, they showed elevated serum and secretory basal IgA levels. LRBA was dispensable for B- and T-cell development, as well as for in vitro B-cell proliferation, survival, isotype switching and plasmablast differentiation. Interestingly, Lrba-/- mice displayed decreased cytotoxic T-lymphocyte-associated protein-4 (CTLA-4) expression by regulatory T cells and activated conventional CD4+ and CD8+ T lymphocytes, reduced frequency of peritoneal B-1a cells along with diminished interleukin-10 production and increased percentages of T follicular helper cells in Peyer's patches, but without developing overt signs of autoimmunity. Our findings expand the role of LRBA in immune regulatory mechanisms previously reported in patients, and suggest a novel role in IgA production that is crucial for the protection of mucosal surfaces and gut-associated immune tolerance. Biallelic mutations in the human lipopolysaccharide responsive beige‐like anchor (LRBA) gene lead to a primary immunodeficiency known as LRBA deficiency, characterized by a broad range of clinical manifestations including autoimmunity, organomegaly, hypogammaglobulinemia and recurrent infections. Considering the phenotypic heterogeneity in patients and the severity of the disease, our aim was to assess the role of LRBA in immune cells and to understand the underlying pathomechanisms through the study of a Lrba knockout (Lrba−/−) mouse model. LRBA‐deficient mice did not show severe clinical or immunological signs of disease, either at steady state under specific‐pathogen‐free conditions, after vaccination with T‐dependent and T‐independent antigens, or in the context of acute infections with lymphocytic choriomeningitis virus (LCMV) or Salmonella Typhimurium. Although Lrba−/− mice were able to produce normal serum immunoglobulin M (IgM) and IgG and to mount a specific immune response after immunization, they showed elevated serum and secretory basal IgA levels. LRBA was dispensable for B‐ and T‐cell development, as well as for in vitro B‐cell proliferation, survival, isotype switching and plasmablast differentiation. Interestingly, Lrba−/− mice displayed decreased cytotoxic T‐lymphocyte‐associated protein‐4 (CTLA‐4) expression by regulatory T cells and activated conventional CD4+ and CD8+ T lymphocytes, reduced frequency of peritoneal B‐1a cells along with diminished interleukin‐10 production and increased percentages of T follicular helper cells in Peyer's patches, but without developing overt signs of autoimmunity. Our findings expand the role of LRBA in immune regulatory mechanisms previously reported in patients, and suggest a novel role in IgA production that is crucial for the protection of mucosal surfaces and gut‐associated immune tolerance. Biallelic mutations in the human lipopolysaccharide responsive beige-like anchor (LRBA) gene lead to a primary immunodeficiency known as LRBA deficiency, characterized by a broad range of clinical manifestations including autoimmunity, organomegaly, hypogammaglobulinemia and recurrent infections. Considering the phenotypic heterogeneity in patients and the severity of the disease, our aim was to assess the role of LRBA in immune cells and to understand the underlying pathomechanisms through the study of a Lrba knockout (Lrba ) mouse model. LRBA-deficient mice did not show severe clinical or immunological signs of disease, either at steady state under specific-pathogen-free conditions, after vaccination with T-dependent and T-independent antigens, or in the context of acute infections with lymphocytic choriomeningitis virus (LCMV) or Salmonella Typhimurium. Although Lrba mice were able to produce normal serum immunoglobulin M (IgM) and IgG and to mount a specific immune response after immunization, they showed elevated serum and secretory basal IgA levels. LRBA was dispensable for B- and T-cell development, as well as for in vitro B-cell proliferation, survival, isotype switching and plasmablast differentiation. Interestingly, Lrba mice displayed decreased cytotoxic T-lymphocyte-associated protein-4 (CTLA-4) expression by regulatory T cells and activated conventional CD4 and CD8 T lymphocytes, reduced frequency of peritoneal B-1a cells along with diminished interleukin-10 production and increased percentages of T follicular helper cells in Peyer's patches, but without developing overt signs of autoimmunity. Our findings expand the role of LRBA in immune regulatory mechanisms previously reported in patients, and suggest a novel role in IgA production that is crucial for the protection of mucosal surfaces and gut-associated immune tolerance. |
| Author | Grassi, Fabio Kögl, Tamara Jäger, Fiona Proietti, Michele Neumann, Julika Gámez‐Díaz, Laura Perruzza, Lisa Soulas‐Sprauel, Pauline Jung, Sophie Felber, Felicitas Grimbacher, Bodo Kilimann, Manfred Aichele, Peter |
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| Cites_doi | 10.1007/s10875-015-0224-7 10.1371/journal.pone.0139729 10.1016/j.jaci.2012.05.043 10.1016/0092-8674(93)80068-P 10.1128/IAI.71.5.2839-2858.2003 10.1038/nm.3746 10.1016/j.jaci.2012.07.035 10.1126/science.270.5238.985 10.1210/jc.2015-3382 10.1016/S0952-7915(00)00204-1 10.1016/0092-8674(92)90030-G 10.4049/jimmunol.166.7.4586 10.1126/science.1202947 10.1038/s41598-017-08543-4 10.1097/MIB.0000000000000266 10.1038/ng.884 10.1093/oxfordjournals.jhered.a107620 10.1111/cei.12008 10.1172/JCI20096 10.1084/jem.188.12.2381 10.1038/nri3007 10.1126/science.aaa1663 10.1016/j.clim.2016.03.006 10.1016/j.immuni.2015.04.005 10.1038/295240a0 10.1038/sj.onc.1207567 10.1016/j.jaci.2014.10.048 10.1038/srep36568 10.1016/j.coi.2012.04.005 10.1182/blood-2016-04-712612 10.1093/intimm/dxn031 10.1111/tra.12069 10.1002/eji.1830030911 10.1016/j.bbmt.2008.10.026 10.18632/oncotarget.8853 10.3389/fimmu.2017.00052 10.1016/0092-8674(92)90029-C 10.1007/s10875-015-0220-y 10.1084/jem.20061041 10.1038/icb.1995.66 10.1016/S1074-7613(01)00129-7 10.1126/science.290.5489.89 10.1038/nri2901 10.1016/j.jaci.2015.09.025 10.1016/j.jaci.2014.10.019 10.1016/j.autrev.2005.10.007 10.1016/j.immuni.2008.03.017 10.1038/ni.1662 10.1016/0092-8674(95)90415-8 10.1038/ni.1820 10.1016/j.immuni.2005.04.011 10.1093/intimm/5.10.1317 10.1016/j.ajhg.2012.04.015 10.1016/S1074-7613(00)80406-9 10.1189/jlb.0506364 |
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| References | 1995; 73 2017; 8 2004; 23 2008; 9 2016; 101 2000; 95 2011; 11 2015; 349 2016; 36 2005; 23 2000; 290 1993; 5 1997; 7 2014; 20 2012; 130 2013; 14 2009; 10 2015; 135 2015; 42 1993; 75 2008; 28 1967; 58 2008; 20 2012; 24 2006; 203 2001; 13 2001; 14 1982; 295 2009; 15 2001; 166 1979; 123 2015; 10 2016; 168 2006; 5 2016; 128 2003; 71 2011; 332 1995; 270 2016; 6 2016; 7 2012; 90 1995; 80 2004; 113 2015; 21 2011; 43 2007; 81 2017 1992; 68 2016; 137 1998; 188 2013; 171 1973; 3 e_1_2_7_5_1 e_1_2_7_3_1 e_1_2_7_9_1 e_1_2_7_7_1 Certain S (e_1_2_7_16_1) 2000; 95 e_1_2_7_19_1 e_1_2_7_17_1 e_1_2_7_15_1 e_1_2_7_41_1 e_1_2_7_1_1 e_1_2_7_13_1 e_1_2_7_43_1 e_1_2_7_11_1 e_1_2_7_47_1 e_1_2_7_26_1 e_1_2_7_49_1 e_1_2_7_28_1 e_1_2_7_50_1 e_1_2_7_25_1 e_1_2_7_31_1 Roder JC (e_1_2_7_45_1) 1979; 123 e_1_2_7_52_1 e_1_2_7_23_1 e_1_2_7_33_1 e_1_2_7_54_1 e_1_2_7_21_1 e_1_2_7_35_1 e_1_2_7_56_1 e_1_2_7_37_1 e_1_2_7_39_1 e_1_2_7_6_1 e_1_2_7_4_1 e_1_2_7_8_1 e_1_2_7_18_1 e_1_2_7_40_1 e_1_2_7_2_1 e_1_2_7_14_1 e_1_2_7_42_1 e_1_2_7_12_1 e_1_2_7_44_1 e_1_2_7_10_1 e_1_2_7_46_1 e_1_2_7_48_1 e_1_2_7_27_1 e_1_2_7_29_1 e_1_2_7_51_1 e_1_2_7_30_1 e_1_2_7_53_1 e_1_2_7_24_1 e_1_2_7_32_1 e_1_2_7_55_1 e_1_2_7_22_1 e_1_2_7_34_1 e_1_2_7_57_1 e_1_2_7_20_1 e_1_2_7_36_1 e_1_2_7_38_1 10648412 - Blood. 2000 Feb 1;95(3):979-83 22721650 - J Allergy Clin Immunol. 2012 Aug;130(2):481-8.e2 18836449 - Nat Immunol. 2008 Nov;9(11):1307-15 9858525 - J Exp Med. 1998 Dec 21;188(12):2381-6 26745254 - J Clin Endocrinol Metab. 2016 Mar;101(3):898-904 21474713 - Science. 2011 Apr 29;332(6029):600-3 26768763 - J Allergy Clin Immunol. 2016 Jan;137(1):223-230 9430233 - Immunity. 1997 Dec;7(6):885-95 19898472 - Nat Immunol. 2009 Dec;10(12):1283-91 22608502 - Am J Hum Genet. 2012 Jun 8;90(6):986-1001 18375938 - Int Immunol. 2008 Jun;20(6):729-37 22981790 - J Allergy Clin Immunol. 2012 Dec;130(6):1428-32 16039575 - Immunity. 2005 Jul;23(1):7-18 27105495 - Oncotarget. 2016 May 10;7(19):26992-7006 26206937 - Science. 2015 Jul 24;349(6246):436-40 19135937 - Biol Blood Marrow Transplant. 2009 Jan;15(1):1-11 7481803 - Science. 1995 Nov 10;270(5238):985-8 7505612 - Int Immunol. 1993 Oct;5(10):1317-27 21765413 - Nat Genet. 2011 Jul 17;43(8):738-40 8595920 - Immunol Cell Biol. 1995 Oct;73(5):425-32 22560294 - Curr Opin Immunol. 2012 Aug;24(4):482-7 17060475 - J Exp Med. 2006 Oct 30;203(11):2541-50 25479458 - Inflamm Bowel Dis. 2015 Jan;21(1):40-7 25329329 - Nat Med. 2014 Dec;20(12 ):1410-1416 16890894 - Autoimmun Rev. 2006 Jul;5(6):403-8 25902480 - Immunity. 2015 Apr 21;42(4):607-12 26707784 - J Clin Immunol. 2016 Jan;36(1):33-45 27418640 - Blood. 2016 Aug 25;128(8):1037-42 8402911 - Cell. 1993 Oct 22;75(2):263-74 26686526 - J Clin Immunol. 2016 Jan;36(1):8-11 27057999 - Clin Immunol. 2016 Jul;168:88-93 7834752 - Cell. 1995 Jan 27;80(2):321-30 25539626 - J Allergy Clin Immunol. 2015 May;135(5):1384-90.e1-8 489978 - J Immunol. 1979 Nov;123(5):2168-73 21681197 - Nat Rev Immunol. 2011 Jun 17;11(7):445-56 4203294 - Eur J Immunol. 1973 Sep;3(9):580-4 15064745 - Oncogene. 2004 May 20;23(23):4089-97 23379434 - Clin Exp Immunol. 2013 Mar;171(3):278-82 28814779 - Sci Rep. 2017 Aug 16;7(1):8409 1547488 - Cell. 1992 Mar 6;68(5):869-77 23521701 - Traffic. 2013 Jul;14(7):749-66 1547487 - Cell. 1992 Mar 6;68(5):855-67 11254716 - J Immunol. 2001 Apr 1;166(7):4586-95 11021805 - Science. 2000 Oct 6;290(5489):89-92 12704158 - Infect Immun. 2003 May;71(5):2839-58 6977094 - Nature. 1982 Jan 21;295(5846):240-1 17369496 - J Leukoc Biol. 2007 Jun;81(6):1386-94 26448644 - PLoS One. 2015 Oct 08;10(10):e0139729 11371363 - Immunity. 2001 May;14(5):617-29 18482568 - Immunity. 2008 May;28(5):639-50 11228413 - Curr Opin Immunol. 2001 Apr;13(2):195-201 25468195 - J Allergy Clin Immunol. 2015 Jan;135(1):217-27 28197149 - Front Immunol. 2017 Jan 31;8:52 15067315 - J Clin Invest. 2004 Mar;113(6):826-35 5590154 - J Hered. 1967 Nov-Dec;58(6):299-300 27824136 - Sci Rep. 2016 Nov 08;6:36568 21151033 - Nat Rev Immunol. 2011 Jan;11(1):34-46 |
| References_xml | – volume: 130 start-page: 1428 year: 2012 end-page: 1432 article-title: LRBA gene deletion in a patient presenting with autoimmunity without hypogammaglobulinemia publication-title: J Allergy Clin Immunol – volume: 135 start-page: 217 year: 2015 end-page: 227 article-title: Regulatory T‐cell deficiency and immune dysregulation, polyendocrinopathy, enteropathy, X‐linked‐like disorder caused by loss‐of‐function mutations in LRBA publication-title: J Allergy Clin Immunol – volume: 68 start-page: 855 year: 1992 end-page: 867 article-title: RAG‐2‐deficient mice lack mature lymphocytes owing to inability to initiate V(D)J rearrangement publication-title: Cell – volume: 137 start-page: 223 year: 2016 end-page: 230 article-title: The extended phenotype of LPS‐responsive beige‐like anchor protein (LRBA) deficiency publication-title: J Allergy Clin Immunol – volume: 36 start-page: 33 year: 2016 end-page: 45 article-title: Spectrum of Phenotypes associated with mutations in LRBA publication-title: J Clin Immunol – volume: 11 start-page: 34 year: 2011 end-page: 46 article-title: The double life of a B‐1 cell: self‐reactivity selects for protective effector functions publication-title: Nat Rev Immunol – volume: 80 start-page: 321 year: 1995 end-page: 330 article-title: Targeted disruption of the p50 subunit of NF‐κB leads to multifocal defects in immune responses publication-title: Cell – volume: 135 start-page: 1384 year: 2015 end-page: 1390.e1–8 article-title: Long‐term remission after allogeneic hematopoietic stem cell transplantation in LPS‐responsive beige‐like anchor (LRBA) deficiency publication-title: J Allergy Clin Immunol – volume: 171 start-page: 278 year: 2013 end-page: 282 article-title: Circulating phenotypic B‐1 cells are decreased in common variable immunodeficiency and correlate with immunoglobulin M levels publication-title: Clin Exp Immunol – volume: 5 start-page: 403 year: 2006 end-page: 408 article-title: Role of B‐1a cells in autoimmunity publication-title: Autoimmun Rev – volume: 168 start-page: 88 year: 2016 end-page: 93 article-title: LRBA deficiency with autoimmunity and early onset chronic erosive polyarthritis publication-title: Clin Immunol (Orlando, FL) – volume: 3 start-page: 580 year: 1973 end-page: 584 article-title: Mesenteric lymph as a major source of serum IgA in guinea pigs and rats publication-title: Eur J Immunol – volume: 20 start-page: 1410 year: 2014 end-page: 1416 article-title: Autosomal dominant immune dysregulation syndrome in humans with CTLA4 mutations publication-title: Nat Med – volume: 6 start-page: 36568 year: 2016 article-title: LRBA is essential for allogeneic responses in bone marrow transplantation publication-title: Sci Rep – volume: 7 start-page: 26992 year: 2016 end-page: 27006 article-title: Dysregulation of peritoneal cavity B1a cells and murine primary biliary cholangitis publication-title: Oncotarget – volume: 7 start-page: 885 year: 1997 end-page: 895 article-title: Lymphoproliferation in CTLA‐4‐deficient mice is mediated by costimulation‐dependent activation of CD4 T cells publication-title: Immunity – volume: 270 start-page: 985 year: 1995 end-page: 988 article-title: Lymphoproliferative disorders with early lethality in mice deficient in Ctla‐4 publication-title: Science – volume: 68 start-page: 869 year: 1992 end-page: 877 article-title: RAG‐1‐deficient mice have no mature B and T lymphocytes publication-title: Cell – volume: 21 start-page: 40 year: 2015 end-page: 47 article-title: Atypical manifestation of LRBA deficiency with predominant IBD‐like phenotype publication-title: Inflamm Bowel Dis – volume: 23 start-page: 7 year: 2005 end-page: 18 article-title: B‐1a and B‐1b cells exhibit distinct developmental requirements and have unique functional roles in innate and adaptive immunity to S. pneumoniae publication-title: Immunity – volume: 332 start-page: 600 year: 2011 end-page: 603 article-title: Trans‐endocytosis of CD80 and CD86: a molecular basis for the cell‐extrinsic function of CTLA‐4 publication-title: Science – volume: 23 start-page: 4089 year: 2004 end-page: 4097 article-title: Deregulated expression of LRBA facilitates cancer cell growth publication-title: Oncogene – volume: 295 start-page: 240 year: 1982 end-page: 241 article-title: Defective T‐cell response in beige mutant mice publication-title: Nature – volume: 101 start-page: 898 year: 2016 end-page: 904 article-title: Infancy‐onset T1DM, short stature, and severe immunodysregulation in two siblings with a homozygous LRBA mutation publication-title: J Clin Endocrinol Metab – volume: 290 start-page: 89 year: 2000 end-page: 92 article-title: T‐Independent immune response: new aspects of B cell biology publication-title: Science – volume: 10 start-page: e0139729 year: 2015 article-title: Age‐dependent defects of regulatory B cells in Wiskott–Aldrich Syndrome gene knockout mice publication-title: PLoS ONE – volume: 75 start-page: 263 year: 1993 end-page: 274 article-title: Interleukin‐10‐deficient mice develop chronic enterocolitis publication-title: Cell – volume: 81 start-page: 1386 year: 2007 end-page: 1394 article-title: Changes of CD4 CD25 Foxp3 regulatory T cells in aged Balb/c mice publication-title: J Leukoc Biol – volume: 188 start-page: 2381 year: 1998 end-page: 2386 article-title: A critical role of natural immunoglobulin M in immediate defense against systemic bacterial infection publication-title: J Exp Med – volume: 58 start-page: 299 year: 1967 end-page: 300 article-title: Giant granules in leukocytes of the beige mouse publication-title: J Hered – volume: 20 start-page: 729 year: 2008 end-page: 737 article-title: Regulatory role of B‐1 B cells in chronic colitis publication-title: Int Immunol – volume: 36 start-page: 8 year: 2016 end-page: 11 article-title: A successful hSCT in a girl with novel LRBA mutation with refractory celiac disease publication-title: J Clin Immunol – volume: 203 start-page: 2541 year: 2006 end-page: 2550 article-title: Regulation of B1 cell migration by signals through Toll‐like receptors publication-title: J Exp Med – volume: 95 start-page: 979 year: 2000 end-page: 983 article-title: Protein truncation test of LYST reveals heterogenous mutations in patients with Chediak–Higashi syndrome publication-title: Blood – volume: 24 start-page: 482 year: 2012 end-page: 487 article-title: Homeostasis and function of regulatory T cells in aging publication-title: Curr Opin Immunol – volume: 128 start-page: 1037 year: 2016 end-page: 1042 article-title: CHAI and LATAIE: new genetic diseases of CTLA‐4 checkpoint insufficiency publication-title: Blood – volume: 9 start-page: 1307 year: 2008 end-page: 1315 article-title: The actin regulator coronin 1A is mutant in a thymic egress‐deficient mouse strain and in a patient with severe combined immunodeficiency publication-title: Nat Immunol – volume: 71 start-page: 2839 year: 2003 end-page: 2858 article-title: Pretreatment of mice with streptomycin provides a serovar Typhimurium colitis model that allows analysis of both pathogen and host publication-title: Infect Immun – volume: 8 start-page: 52 year: 2017 article-title: Treatment of infantile inflammatory bowel disease and autoimmunity by allogeneic stem cell transplantation in LPS‐responsive Beige‐like anchor deficiency publication-title: Front Immunol – volume: 5 start-page: 1317 year: 1993 end-page: 1327 article-title: Evidence that intestinal IgA plasma cells in mu, kappa transgenic mice are derived from B‐1 (Ly‐1 B) cells publication-title: Int Immunol – volume: 123 start-page: 2168 year: 1979 end-page: 2173 article-title: The beige mutation in the mouse. I. A stem cell predetermined impairment in natural killer cell function publication-title: J Immunol (Baltimore, MD, 1950) – volume: 349 start-page: 436 year: 2015 end-page: 440 article-title: Autoimmune disease. Patients with LRBA deficiency show CTLA4 loss and immune dysregulation responsive to abatacept therapy publication-title: Science – volume: 13 start-page: 195 year: 2001 end-page: 201 article-title: B1 cells: similarities and differences with other B cell subsets publication-title: Curr Opin Immunol – volume: 14 start-page: 617 year: 2001 end-page: 629 article-title: Marginal zone and B1 B cells unite in the early response against T‐independent blood‐borne particulate antigens publication-title: Immunity – volume: 113 start-page: 826 year: 2004 end-page: 835 article-title: Dysregulated LIGHT expression on T cells mediates intestinal inflammation and contributes to IgA nephropathy publication-title: J Clin Invest – volume: 28 start-page: 639 year: 2008 end-page: 650 article-title: A regulatory B cell subset with a unique CD1dhiCD5 phenotype controls T cell‐dependent inflammatory responses publication-title: Immunity – volume: 10 start-page: 1283 year: 2009 end-page: 1291 article-title: Dock8 mutations cripple B cell immunological synapses, germinal centers and long‐lived antibody production publication-title: Nat Immunol – volume: 130 start-page: 481 year: 2012 end-page: 488.e2 article-title: LPS‐responsive beige‐like anchor (LRBA) gene mutation in a family with inflammatory bowel disease and combined immunodeficiency publication-title: J Allergy Clin Immunol – volume: 14 start-page: 749 year: 2013 end-page: 766 article-title: The BEACH Is Hot: a LYST of emerging roles for BEACH‐domain containing proteins in human disease publication-title: Traffic – volume: 166 start-page: 4586 year: 2001 end-page: 4595 article-title: Identification of a novel lipopolysaccharide‐inducible gene with key features of both A kinase anchor proteins and chs1/beige proteins publication-title: J Immunol (Baltimore, Md, 1950) – volume: 43 start-page: 738 year: 2011 end-page: 740 article-title: Mutations in NBEAL2, encoding a BEACH protein, cause gray platelet syndrome publication-title: Nat Genet – year: 2017 article-title: The BEACH protein LRBA promotes the localization of the heterotrimeric G‐protein golf to olfactory cilia publication-title: Sci Rep – volume: 15 start-page: 1 year: 2009 end-page: 11 article-title: A non‐leaky Artemis‐deficient mouse that accurately models the human severe combined immune deficiency phenotype, including resistance to hematopoietic stem cell transplantation publication-title: Biol Blood Marrow Transplant J – volume: 11 start-page: 445 year: 2011 end-page: 456 article-title: The light and dark sides of intestinal intraepithelial lymphocytes publication-title: Nat Rev Immunol – volume: 90 start-page: 986 year: 2012 end-page: 1001 article-title: Deleterious mutations in LRBA are associated with a syndrome of immune deficiency and autoimmunity publication-title: Am J Hum Genet – volume: 42 start-page: 607 year: 2015 end-page: 612 article-title: Regulatory B cells: origin, phenotype, and function publication-title: Immunity – volume: 73 start-page: 425 year: 1995 end-page: 432 article-title: Peritoneal cavity CD5 (Bla) B cells: cytokine induced IgA secretion and homing to intestinal lamina propria in SCID mice publication-title: Immunol Cell Biol – ident: e_1_2_7_2_1 doi: 10.1007/s10875-015-0224-7 – ident: e_1_2_7_35_1 doi: 10.1371/journal.pone.0139729 – ident: e_1_2_7_5_1 doi: 10.1016/j.jaci.2012.05.043 – ident: e_1_2_7_52_1 doi: 10.1016/0092-8674(93)80068-P – ident: e_1_2_7_28_1 doi: 10.1128/IAI.71.5.2839-2858.2003 – ident: e_1_2_7_19_1 doi: 10.1038/nm.3746 – ident: e_1_2_7_6_1 doi: 10.1016/j.jaci.2012.07.035 – ident: e_1_2_7_55_1 doi: 10.1126/science.270.5238.985 – ident: e_1_2_7_10_1 doi: 10.1210/jc.2015-3382 – volume: 95 start-page: 979 year: 2000 ident: e_1_2_7_16_1 article-title: Protein truncation test of LYST reveals heterogenous mutations in patients with Chediak–Higashi syndrome publication-title: Blood – ident: e_1_2_7_30_1 doi: 10.1016/S0952-7915(00)00204-1 – ident: e_1_2_7_48_1 doi: 10.1016/0092-8674(92)90030-G – ident: e_1_2_7_14_1 doi: 10.4049/jimmunol.166.7.4586 – ident: e_1_2_7_18_1 doi: 10.1126/science.1202947 – ident: e_1_2_7_57_1 doi: 10.1038/s41598-017-08543-4 – ident: e_1_2_7_12_1 doi: 10.1097/MIB.0000000000000266 – ident: e_1_2_7_17_1 doi: 10.1038/ng.884 – ident: e_1_2_7_44_1 doi: 10.1093/oxfordjournals.jhered.a107620 – ident: e_1_2_7_36_1 doi: 10.1111/cei.12008 – ident: e_1_2_7_42_1 doi: 10.1172/JCI20096 – ident: e_1_2_7_31_1 doi: 10.1084/jem.188.12.2381 – ident: e_1_2_7_27_1 doi: 10.1038/nri3007 – ident: e_1_2_7_8_1 doi: 10.1126/science.aaa1663 – ident: e_1_2_7_7_1 doi: 10.1016/j.clim.2016.03.006 – ident: e_1_2_7_34_1 doi: 10.1016/j.immuni.2015.04.005 – ident: e_1_2_7_46_1 doi: 10.1038/295240a0 – ident: e_1_2_7_43_1 doi: 10.1038/sj.onc.1207567 – volume: 123 start-page: 2168 year: 1979 ident: e_1_2_7_45_1 article-title: The beige mutation in the mouse. I. A stem cell predetermined impairment in natural killer cell function publication-title: J Immunol (Baltimore, MD, 1950) – ident: e_1_2_7_11_1 doi: 10.1016/j.jaci.2014.10.048 – ident: e_1_2_7_56_1 doi: 10.1038/srep36568 – ident: e_1_2_7_21_1 doi: 10.1016/j.coi.2012.04.005 – ident: e_1_2_7_20_1 doi: 10.1182/blood-2016-04-712612 – ident: e_1_2_7_24_1 doi: 10.1093/intimm/dxn031 – ident: e_1_2_7_15_1 doi: 10.1111/tra.12069 – ident: e_1_2_7_41_1 doi: 10.1002/eji.1830030911 – ident: e_1_2_7_50_1 doi: 10.1016/j.bbmt.2008.10.026 – ident: e_1_2_7_26_1 doi: 10.18632/oncotarget.8853 – ident: e_1_2_7_13_1 doi: 10.3389/fimmu.2017.00052 – ident: e_1_2_7_49_1 doi: 10.1016/0092-8674(92)90029-C – ident: e_1_2_7_9_1 doi: 10.1007/s10875-015-0220-y – ident: e_1_2_7_40_1 doi: 10.1084/jem.20061041 – ident: e_1_2_7_38_1 doi: 10.1038/icb.1995.66 – ident: e_1_2_7_29_1 doi: 10.1016/S1074-7613(01)00129-7 – ident: e_1_2_7_37_1 doi: 10.1126/science.290.5489.89 – ident: e_1_2_7_23_1 doi: 10.1038/nri2901 – ident: e_1_2_7_3_1 doi: 10.1016/j.jaci.2015.09.025 – ident: e_1_2_7_4_1 doi: 10.1016/j.jaci.2014.10.019 – ident: e_1_2_7_25_1 doi: 10.1016/j.autrev.2005.10.007 – ident: e_1_2_7_33_1 doi: 10.1016/j.immuni.2008.03.017 – ident: e_1_2_7_51_1 doi: 10.1038/ni.1662 – ident: e_1_2_7_53_1 doi: 10.1016/0092-8674(95)90415-8 – ident: e_1_2_7_47_1 doi: 10.1038/ni.1820 – ident: e_1_2_7_32_1 doi: 10.1016/j.immuni.2005.04.011 – ident: e_1_2_7_39_1 doi: 10.1093/intimm/5.10.1317 – ident: e_1_2_7_1_1 doi: 10.1016/j.ajhg.2012.04.015 – ident: e_1_2_7_54_1 doi: 10.1016/S1074-7613(00)80406-9 – ident: e_1_2_7_22_1 doi: 10.1189/jlb.0506364 – reference: 5590154 - J Hered. 1967 Nov-Dec;58(6):299-300 – reference: 9430233 - Immunity. 1997 Dec;7(6):885-95 – reference: 489978 - J Immunol. 1979 Nov;123(5):2168-73 – reference: 25468195 - J Allergy Clin Immunol. 2015 Jan;135(1):217-27 – reference: 10648412 - Blood. 2000 Feb 1;95(3):979-83 – reference: 8402911 - Cell. 1993 Oct 22;75(2):263-74 – reference: 1547487 - Cell. 1992 Mar 6;68(5):855-67 – reference: 16890894 - Autoimmun Rev. 2006 Jul;5(6):403-8 – reference: 15067315 - J Clin Invest. 2004 Mar;113(6):826-35 – reference: 21765413 - Nat Genet. 2011 Jul 17;43(8):738-40 – reference: 8595920 - Immunol Cell Biol. 1995 Oct;73(5):425-32 – reference: 26448644 - PLoS One. 2015 Oct 08;10(10):e0139729 – reference: 23521701 - Traffic. 2013 Jul;14(7):749-66 – reference: 18482568 - Immunity. 2008 May;28(5):639-50 – reference: 6977094 - Nature. 1982 Jan 21;295(5846):240-1 – reference: 25329329 - Nat Med. 2014 Dec;20(12 ):1410-1416 – reference: 19135937 - Biol Blood Marrow Transplant. 2009 Jan;15(1):1-11 – reference: 26745254 - J Clin Endocrinol Metab. 2016 Mar;101(3):898-904 – reference: 16039575 - Immunity. 2005 Jul;23(1):7-18 – reference: 19898472 - Nat Immunol. 2009 Dec;10(12):1283-91 – reference: 26206937 - Science. 2015 Jul 24;349(6246):436-40 – reference: 15064745 - Oncogene. 2004 May 20;23(23):4089-97 – reference: 7505612 - Int Immunol. 1993 Oct;5(10):1317-27 – reference: 27824136 - Sci Rep. 2016 Nov 08;6:36568 – reference: 7834752 - Cell. 1995 Jan 27;80(2):321-30 – reference: 25479458 - Inflamm Bowel Dis. 2015 Jan;21(1):40-7 – reference: 27418640 - Blood. 2016 Aug 25;128(8):1037-42 – reference: 17060475 - J Exp Med. 2006 Oct 30;203(11):2541-50 – reference: 22608502 - Am J Hum Genet. 2012 Jun 8;90(6):986-1001 – reference: 12704158 - Infect Immun. 2003 May;71(5):2839-58 – reference: 28197149 - Front Immunol. 2017 Jan 31;8:52 – reference: 27105495 - Oncotarget. 2016 May 10;7(19):26992-7006 – reference: 23379434 - Clin Exp Immunol. 2013 Mar;171(3):278-82 – reference: 11228413 - Curr Opin Immunol. 2001 Apr;13(2):195-201 – reference: 1547488 - Cell. 1992 Mar 6;68(5):869-77 – reference: 4203294 - Eur J Immunol. 1973 Sep;3(9):580-4 – reference: 11254716 - J Immunol. 2001 Apr 1;166(7):4586-95 – reference: 17369496 - J Leukoc Biol. 2007 Jun;81(6):1386-94 – reference: 21474713 - Science. 2011 Apr 29;332(6029):600-3 – reference: 22721650 - J Allergy Clin Immunol. 2012 Aug;130(2):481-8.e2 – reference: 7481803 - Science. 1995 Nov 10;270(5238):985-8 – reference: 26768763 - J Allergy Clin Immunol. 2016 Jan;137(1):223-230 – reference: 28814779 - Sci Rep. 2017 Aug 16;7(1):8409 – reference: 18375938 - Int Immunol. 2008 Jun;20(6):729-37 – reference: 22560294 - Curr Opin Immunol. 2012 Aug;24(4):482-7 – reference: 27057999 - Clin Immunol. 2016 Jul;168:88-93 – reference: 9858525 - J Exp Med. 1998 Dec 21;188(12):2381-6 – reference: 25539626 - J Allergy Clin Immunol. 2015 May;135(5):1384-90.e1-8 – reference: 21681197 - Nat Rev Immunol. 2011 Jun 17;11(7):445-56 – reference: 18836449 - Nat Immunol. 2008 Nov;9(11):1307-15 – reference: 21151033 - Nat Rev Immunol. 2011 Jan;11(1):34-46 – reference: 11021805 - Science. 2000 Oct 6;290(5489):89-92 – reference: 26686526 - J Clin Immunol. 2016 Jan;36(1):8-11 – reference: 11371363 - Immunity. 2001 May;14(5):617-29 – reference: 22981790 - J Allergy Clin Immunol. 2012 Dec;130(6):1428-32 – reference: 26707784 - J Clin Immunol. 2016 Jan;36(1):33-45 – reference: 25902480 - Immunity. 2015 Apr 21;42(4):607-12 |
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| Snippet | Biallelic mutations in the human lipopolysaccharide responsive beige‐like anchor (LRBA) gene lead to a primary immunodeficiency known as LRBA deficiency,... Biallelic mutations in the human lipopolysaccharide responsive beige‐like anchor ( LRBA ) gene lead to a primary immunodeficiency known as LRBA deficiency,... Biallelic mutations in the human lipopolysaccharide responsive beige-like anchor (LRBA) gene lead to a primary immunodeficiency known as LRBA deficiency,... |
| SourceID | hal proquest pubmed crossref wiley |
| SourceType | Open Access Repository Aggregation Database Index Database Enrichment Source Publisher |
| StartPage | 789 |
| SubjectTerms | Adaptor Proteins, Signal Transducing - genetics Adaptor Proteins, Signal Transducing - metabolism Animals Antigens Autoimmunity CD4 antigen CD8 antigen Cell proliferation Cell survival CTLA-4 Antigen - genetics CTLA-4 Antigen - metabolism CTLA-4 protein Cytotoxicity Gene Expression Regulation Germinal Center - immunology Helper cells Humans Hypogammaglobulinemia Immunodeficiency Immunoglobulin A Immunoglobulin A - metabolism Immunoglobulin G Immunoglobulin M Immunologic Deficiency Syndromes - genetics Immunological tolerance Immunology Immunoregulation Interleukin 10 Interleukin-10 - metabolism Life Sciences Lipopolysaccharides Lymphocytes B Lymphocytes T Lymphocytic Choriomeningitis - immunology Lymphocytic choriomeningitis virus - immunology Mice Mice, Inbred C57BL Mice, Knockout Mucosal immunity Peritoneum Peyer's patches Phenotypes Primary immunodeficiencies Rodents Salmonella Infections, Animal - immunology Salmonella typhimurium - immunology T-Lymphocytes, Helper-Inducer - immunology T-Lymphocytes, Regulatory - immunology Vaccination |
| Title | Immunological phenotype of the murine Lrba knockout |
| URI | https://onlinelibrary.wiley.com/doi/abs/10.1038%2Ficb.2017.52 https://www.ncbi.nlm.nih.gov/pubmed/28652580 https://www.proquest.com/docview/1949061700 https://www.proquest.com/docview/1914290583 https://hal.science/hal-03415531 |
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