دورية أكاديمية
أعرض في EDS

Patients and mice with deficiency in the SNARE protein SYNTAXIN-11 have a secondary B cell defect.

التفاصيل البيبلوغرافية
العنوان: Patients and mice with deficiency in the SNARE protein SYNTAXIN-11 have a secondary B cell defect.
المؤلفون: Kögl T; Institute for Immunology, Center for Microbiology and Hygiene, Medical Center-University of Freiburg , Freiburg, Germany.; Faculty of Medicine, Institute for Immunodeficiency, Medical Center-University of Freiburg, Freiburg, Germany., Chang HF; Cellular Neurophysiology, Center for Integrative Physiology and Molecular Medicine, Saarland University , Homburg, Germany., Staniek J; Faculty of Medicine, Center for Chronic Immunodeficiency, Medical Center-University of Freiburg, Freiburg, Germany.; Department of Rheumatology and Clinical Immunology, Faculty of Medicine, Medical Center- University of Freiburg, Freiburg, Germany., Chiang SCC; Division of Bone Marrow Transplantation and Immune Deficiency, and Department of Pediatrics, Cincinnati Children's Hospital Medical Center, University of Cincinnati, Cincinnati, OH, USA.; Department of Medicine, Center for Hematology and Regenerative Medicine Huddinge, Karolinska Institute, Karolinska University Hospital Huddinge, Stockholm, Sweden., Thoulass G; Faculty of Medicine, Institute for Immunodeficiency, Medical Center-University of Freiburg, Freiburg, Germany.; Faculty of Medicine, Center for Chronic Immunodeficiency, Medical Center-University of Freiburg, Freiburg, Germany.; Faculty of Biology, Albert-Ludwigs-University of Freiburg, Freiburg, Germany., Lao J; Faculty of Medicine, Institute for Immunodeficiency, Medical Center-University of Freiburg, Freiburg, Germany.; Faculty of Medicine, Center for Chronic Immunodeficiency, Medical Center-University of Freiburg, Freiburg, Germany.; Faculty of Biology, Albert-Ludwigs-University of Freiburg, Freiburg, Germany., Weißert K; Faculty of Medicine, Institute for Immunodeficiency, Medical Center-University of Freiburg, Freiburg, Germany.; Faculty of Medicine, Center for Chronic Immunodeficiency, Medical Center-University of Freiburg, Freiburg, Germany., Dettmer-Monaco V; Faculty of Medicine, Center for Chronic Immunodeficiency, Medical Center-University of Freiburg, Freiburg, Germany.; Faculty of Medicine, Institute for Transfusion Medicine and Gene Therapy-University of Freiburg, Freiburg, Germany., Geiger K; Faculty of Medicine, Center for Chronic Immunodeficiency, Medical Center-University of Freiburg, Freiburg, Germany.; Faculty of Biology, Albert-Ludwigs-University of Freiburg, Freiburg, Germany.; Faculty of Medicine, Institute for Transfusion Medicine and Gene Therapy-University of Freiburg, Freiburg, Germany., Manna PT; Department of Neuroscience and Physiology, University of Gothenburg, Gothenburg, Sweden., Beziat V; Laboratory of Human Genetics of Infectious Diseases, Necker Branch, INSERM, Necker Hospital for Sick Children, Paris, France.; Imagine Institute, University of Paris-Cité , Paris, France.; St. Giles Laboratory of Human Genetics of Infectious Diseases, Rockefeller Branch, The Rockefeller University, New York, NY, USA., Momenilandi M; Laboratory of Human Genetics of Infectious Diseases, Necker Branch, INSERM, Necker Hospital for Sick Children, Paris, France.; Imagine Institute, University of Paris-Cité , Paris, France., Tu SM; Cellular Neurophysiology, Center for Integrative Physiology and Molecular Medicine, Saarland University , Homburg, Germany., Keppler SJ; Division of Rheumatology and Immunology, Medical University of Graz, Graz, Austria., Pattu V; Cellular Neurophysiology, Center for Integrative Physiology and Molecular Medicine, Saarland University , Homburg, Germany., Wolf P; Department of Urology, Faculty of Medicine, Medical Center-University of Freiburg, Freiburg, Germany., Kupferschmid L; Institute of Medical Microbiology and Hygiene, University Medical Center , Freiburg, Germany., Tholen S; Department of Pathology, Institute of Surgical Pathology, University Medical Center, University of Freiburg, Freiburg, Germany., Covill LE; Department of Medicine, Center for Hematology and Regenerative Medicine Huddinge, Karolinska Institute, Karolinska University Hospital Huddinge, Stockholm, Sweden., Ebert K; Institute for Immunology, Center for Microbiology and Hygiene, Medical Center-University of Freiburg , Freiburg, Germany., Straub T; Institute for Immunology, Center for Microbiology and Hygiene, Medical Center-University of Freiburg , Freiburg, Germany., Groß M; Faculty of Medicine, Institute for Immunodeficiency, Medical Center-University of Freiburg, Freiburg, Germany.; Faculty of Medicine, Center for Chronic Immunodeficiency, Medical Center-University of Freiburg, Freiburg, Germany., Gather R; Faculty of Medicine, Institute for Immunodeficiency, Medical Center-University of Freiburg, Freiburg, Germany.; Faculty of Medicine, Center for Chronic Immunodeficiency, Medical Center-University of Freiburg, Freiburg, Germany., Engel H; Institute for Immunology, Center for Microbiology and Hygiene, Medical Center-University of Freiburg , Freiburg, Germany., Salzer U; Faculty of Medicine, Center for Chronic Immunodeficiency, Medical Center-University of Freiburg, Freiburg, Germany.; Department of Rheumatology and Clinical Immunology, Faculty of Medicine, Medical Center- University of Freiburg, Freiburg, Germany., Schell C; Department of Pathology, Institute of Surgical Pathology, University Medical Center, University of Freiburg, Freiburg, Germany., Maier S; Division of Pediatric Stem Cell Transplantation and Immunology, University Medical Center Hamburg-Eppendorf, Hamburg, Germany., Lehmberg K; Division of Pediatric Stem Cell Transplantation and Immunology, University Medical Center Hamburg-Eppendorf, Hamburg, Germany., Cornu TI; Faculty of Medicine, Center for Chronic Immunodeficiency, Medical Center-University of Freiburg, Freiburg, Germany.; Faculty of Medicine, Institute for Transfusion Medicine and Gene Therapy-University of Freiburg, Freiburg, Germany., Pircher H; Institute for Immunology, Center for Microbiology and Hygiene, Medical Center-University of Freiburg , Freiburg, Germany., Shahrooei M; Department of Microbiology, Immunology, and Transplantation, Clinical and Diagnostic Immunology, KU Leuven, Leuven, Belgium.; Dr. Shahrooei Laboratory, Tehran University of Medical Sciences, Tehran, Iran., Parvaneh N; Division of Allergy and Clinical Immunology, Department of Pediatrics, Tehran University of Medical Sciences, Tehran, Iran.; Research Center for Immunodeficiencies, Tehran University of Medical Sciences , Tehran, Iran., Elling R; Faculty of Medicine, Center for Chronic Immunodeficiency, Medical Center-University of Freiburg, Freiburg, Germany.; Faculty for Medicine, Center for Pediatrics and Adolescent Medicine, Medical Center-University of Freiburg, Freiburg, Germany., Rizzi M; Faculty of Medicine, Center for Chronic Immunodeficiency, Medical Center-University of Freiburg, Freiburg, Germany.; Department of Rheumatology and Clinical Immunology, Faculty of Medicine, Medical Center- University of Freiburg, Freiburg, Germany.; Division of Clinical and Experimental Immunology, Institute of Immunology, Center for Pathophysiology, Infectiology and Immunology, Medical University of Vienna, Vienna, Austria.; Centre for Integrative Biological Signalling Studies, University of Freiburg , Freiburg, Germany.; Faculty of Medicine, Clinical Immunology, Medical Center-University of Freiburg, Freiburg, Germany., Bryceson YT; Department of Medicine, Center for Hematology and Regenerative Medicine Huddinge, Karolinska Institute, Karolinska University Hospital Huddinge, Stockholm, Sweden.; Division of Clinical Immunology and Transfusion Medicine, Karolinska University Hospital Huddinge, Stockholm, Sweden.; Broegelmann Laboratory, Department of Clinical Sciences, University of Bergen, Bergen, Norway., Ehl S; Faculty of Medicine, Institute for Immunodeficiency, Medical Center-University of Freiburg, Freiburg, Germany.; Faculty of Medicine, Center for Chronic Immunodeficiency, Medical Center-University of Freiburg, Freiburg, Germany., Aichele P; Faculty of Medicine, Institute for Immunodeficiency, Medical Center-University of Freiburg, Freiburg, Germany.; Faculty of Medicine, Center for Chronic Immunodeficiency, Medical Center-University of Freiburg, Freiburg, Germany., Ammann S; Faculty of Medicine, Institute for Immunodeficiency, Medical Center-University of Freiburg, Freiburg, Germany.; Faculty of Medicine, Center for Chronic Immunodeficiency, Medical Center-University of Freiburg, Freiburg, Germany.
المصدر: The Journal of experimental medicine [J Exp Med] 2024 Jul 01; Vol. 221 (7). Date of Electronic Publication: 2024 May 09.
نوع المنشور: Journal Article; Research Support, Non-U.S. Gov't
اللغة: English
بيانات الدورية: Publisher: Rockefeller University Press Country of Publication: United States NLM ID: 2985109R Publication Model: Print-Electronic Cited Medium: Internet ISSN: 1540-9538 (Electronic) Linking ISSN: 00221007 NLM ISO Abbreviation: J Exp Med Subsets: MEDLINE
أسماء مطبوعة: Original Publication: New York, NY : Rockefeller University Press
مواضيع طبية MeSH: Qa-SNARE Proteins*/metabolism , Qa-SNARE Proteins*/genetics , B-Lymphocytes*/immunology , B-Lymphocytes*/metabolism , CD4-Positive T-Lymphocytes*/immunology , CD4-Positive T-Lymphocytes*/metabolism, Animals ; Mice ; Humans ; Lymphohistiocytosis, Hemophagocytic/immunology ; Lymphohistiocytosis, Hemophagocytic/genetics ; Lymphohistiocytosis, Hemophagocytic/metabolism ; Mice, Knockout ; Mice, Inbred C57BL ; Female ; Male ; Germinal Center/immunology ; Germinal Center/metabolism ; Immunity, Humoral ; Exocytosis
مستخلص: SYNTAXIN-11 (STX11) is a SNARE protein that mediates the fusion of cytotoxic granules with the plasma membrane at the immunological synapses of CD8 T or NK cells. Autosomal recessive inheritance of deleterious STX11 variants impairs cytotoxic granule exocytosis, causing familial hemophagocytic lymphohistiocytosis type 4 (FHL-4). In several FHL-4 patients, we also observed hypogammaglobulinemia, elevated frequencies of naive B cells, and increased double-negative DN2:DN1 B cell ratios, indicating a hitherto unrecognized role of STX11 in humoral immunity. Detailed analysis of Stx11-deficient mice revealed impaired CD4 T cell help for B cells, associated with disrupted germinal center formation, reduced isotype class switching, and low antibody avidity. Mechanistically, Stx11-/- CD4 T cells exhibit impaired membrane fusion leading to reduced CD107a and CD40L surface mobilization and diminished IL-2 and IL-10 secretion. Our findings highlight a critical role of STX11 in SNARE-mediated membrane trafficking and vesicle exocytosis in CD4 T cells, important for successful CD4 T cell-B cell interactions. Deficiency in STX11 impairs CD4 T cell-dependent B cell differentiation and humoral responses.
(© 2024 Kögl et al.)
التعليقات: Erratum in: J Exp Med. 2024 Jul 1;221(7):e2022112205142024c. doi: 10.1084/jem.2022112205142024c. (PMID: 38771261)
References: Blood. 2012 Jun 21;119(25):6016-24. (PMID: 22451424)
J Exp Med. 1981 Jan 1;153(1):1-12. (PMID: 6969777)
Br J Haematol. 2002 Jun;117(4):965-72. (PMID: 12060139)
Haematologica. 2010 Dec;95(12):2080-7. (PMID: 20823128)
Cell. 2003 Nov 14;115(4):461-73. (PMID: 14622600)
PLoS Pathog. 2015 Nov 20;11(11):e1005276. (PMID: 26587982)
Blood. 2013 Dec 5;122(24):3940-50. (PMID: 24159173)
Eur J Cell Biol. 2000 Nov;79(11):771-80. (PMID: 11139139)
Nat Cell Biol. 2010 Jan;12(1):19-30; sup pp 1-13. (PMID: 19966785)
Br J Haematol. 2008 Oct;143(1):75-83. (PMID: 18710388)
Immunity. 2022 Feb 8;55(2):272-289.e7. (PMID: 35081372)
FEMS Immunol Med Microbiol. 2004 Jan 15;40(1):63-70. (PMID: 14734188)
J Cell Mol Med. 2012 Jan;16(1):129-41. (PMID: 21342435)
Traffic. 2015 Dec;16(12):1330-41. (PMID: 26771955)
Nat Immunol. 2021 Oct;22(10):1210-1217. (PMID: 34545250)
Immunity. 2003 Nov;19(5):739-48. (PMID: 14614860)
Physiology (Bethesda). 2010 Aug;25(4):218-29. (PMID: 20699468)
EMBO Mol Med. 2009 May;1(2):112-24. (PMID: 20049711)
Proc Natl Acad Sci U S A. 2001 Oct 23;98(22):12614-9. (PMID: 11675497)
Sci Rep. 2019 Sep 30;9(1):13991. (PMID: 31570752)
J Clin Invest. 2009 Dec;119(12):3765-73. (PMID: 19884660)
Immunity. 2011 Oct 28;35(4):622-32. (PMID: 22018472)
Nat Med. 2013 Apr;19(4):494-9. (PMID: 23475201)
Immunity. 2011 Oct 28;35(4):583-95. (PMID: 22018468)
J Pediatr. 2013 Nov;163(5):1253-9. (PMID: 23953723)
Nat Genet. 1996 Nov;14(3):307-11. (PMID: 8896560)
Mol Biol Cell. 2017 Apr 1;28(7):890-897. (PMID: 28179460)
Elife. 2019 Aug 30;8:. (PMID: 31469364)
J Exp Med. 1995 Apr 1;181(4):1293-301. (PMID: 7699321)
Immunity. 2019 Aug 20;51(2):337-350.e7. (PMID: 31375460)
J Exp Med. 2007 Apr 16;204(4):853-63. (PMID: 17420270)
Inflamm Res. 2009 Jul;58(7):407-12. (PMID: 19259622)
Annu Rev Immunol. 2011;29:621-63. (PMID: 21314428)
Blood. 2005 May 1;105(9):3442-8. (PMID: 15632205)
Nat Immunol. 2003 Mar;4(3):261-8. (PMID: 12577056)
Blood. 2007 Sep 15;110(6):1906-15. (PMID: 17525286)
Immunol Cell Biol. 2014 Jan;92(1):34-9. (PMID: 24080769)
Eur J Immunol. 2017 Jan;47(1):41-50. (PMID: 27753080)
Blood. 2023 Dec 7;142(23):1972-1984. (PMID: 37624902)
J Exp Med. 1992 Mar 1;175(3):671-82. (PMID: 1371300)
Hematology. 2005;10 Suppl 1:104-7. (PMID: 16188649)
Clin Exp Immunol. 2000 Mar;119(3):383-9. (PMID: 10691907)
Blood. 1991 Dec 1;78(11):2918-22. (PMID: 1954380)
Nat Biotechnol. 2008 Dec;26(12):1367-72. (PMID: 19029910)
Nature. 2003 Jan 16;421(6920):282-7. (PMID: 12529646)
Front Immunol. 2015 Jan 26;5:691. (PMID: 25674084)
Infect Immun. 1988 Dec;56(12):3064-6. (PMID: 2972626)
Nat Rev Immunol. 2015 Jun;15(6):388-400. (PMID: 25998963)
Nat Rev Immunol. 2009 Jan;9(1):39-46. (PMID: 19079134)
J Immunol. 1998 Nov 1;161(9):4618-26. (PMID: 9794390)
Nat Commun. 2022 Jun 16;13(1):3460. (PMID: 35710644)
Immunity. 2011 Jun 24;34(6):932-46. (PMID: 21636296)
Blood. 2011 Oct 27;118(17):4620-9. (PMID: 21878672)
Blood. 2007 Oct 1;110(7):2520-7. (PMID: 17595332)
Eur J Immunol. 1998 Jan;28(1):390-400. (PMID: 9485218)
Science. 2018 Sep 14;361(6407):. (PMID: 30213884)
J Allergy Clin Immunol. 2014 Jun;133(6):1651-9.e12. (PMID: 24746753)
Science. 2013 Aug 9;341(6146):673-7. (PMID: 23887872)
Am J Hum Genet. 2009 Oct;85(4):482-92. (PMID: 19804848)
Science. 1999 Dec 3;286(5446):1957-9. (PMID: 10583959)
J Virol Methods. 1991 Jun;33(1-2):191-8. (PMID: 1939506)
Curr Opin Pediatr. 2014 Dec;26(6):704-12. (PMID: 25321844)
Nat Immunol. 2012 Mar 11;13(4):405-11. (PMID: 22406686)
J Immunol. 2004 Sep 15;173(6):3647-52. (PMID: 15356109)
Nature. 2021 Mar;591(7850):458-463. (PMID: 33536617)
Front Immunol. 2014 Dec 16;5:630. (PMID: 25566245)
Blood Rev. 2014 Jul;28(4):135-42. (PMID: 24792320)
Eur J Immunol. 2017 Jan;47(1):31-34. (PMID: 28004382)
Mol Pathol. 2003 Oct;56(5):256-62. (PMID: 14514918)
J Cell Biol. 2015 Jul 6;210(1):135-51. (PMID: 26124288)
Blood. 2010 Oct 14;116(15):2635-43. (PMID: 20558610)
Eur J Immunol. 2013 Jan;43(1):194-208. (PMID: 23042080)
Hum Mol Genet. 2005 Mar 15;14(6):827-34. (PMID: 15703195)
Nature. 1996 Jul 18;382(6588):262-5. (PMID: 8717042)
Blood. 2006 Apr 15;107(8):3045-52. (PMID: 16384931)
Proc Natl Acad Sci U S A. 2009 Aug 18;106(33):13945-50. (PMID: 19666484)
J Cell Sci. 1999 Mar;112 ( Pt 6):845-54. (PMID: 10036234)
Curr Biol. 2003 Jan 21;13(2):156-60. (PMID: 12546791)
J Immunol. 2007 Sep 15;179(6):3397-401. (PMID: 17785771)
Trends Immunol. 2007 Nov;28(11):474-81. (PMID: 17962070)
J Clin Immunol. 2015 Jan;35(1):22-5. (PMID: 25491289)
Nat Immunol. 2006 Mar;7(3):247-55. (PMID: 16444260)
J Exp Med. 1991 May 1;173(5):1213-25. (PMID: 1827140)
Br J Haematol. 2001 Sep;114(4):761-9. (PMID: 11564062)
Front Immunol. 2014 Oct 27;5:538. (PMID: 25386181)
Blood. 2013 Jun 27;121(26):5184-91. (PMID: 23660960)
J Exp Med. 2018 Jan 2;215(1):337-355. (PMID: 29212666)
Nat Rev Immunol. 2010 Aug;10(8):568-79. (PMID: 20634814)
Cytometry B Clin Cytom. 2010 Nov;78(6):372-81. (PMID: 20533385)
Am J Hum Genet. 2001 Mar;68(3):590-7. (PMID: 11179007)
Blood. 2016 Jan 28;127(4):426-35. (PMID: 26518437)
Blood. 2004 Aug 1;104(3):735-43. (PMID: 15069016)
Immunity. 2007 Mar;26(3):371-81. (PMID: 17363300)
J Exp Med. 2013 Mar 11;210(3):433-43. (PMID: 23440042)
Blood. 2013 Jan 24;121(4):604-13. (PMID: 23190531)
Ann N Y Acad Sci. 1995 Sep 29;764:27-38. (PMID: 7486535)
Pediatr Blood Cancer. 2010 Jul 15;55(1):134-40. (PMID: 20486178)
Immunity. 2012 May 25;36(5):847-56. (PMID: 22464171)
J Exp Med. 2017 Nov 6;214(11):3381-3398. (PMID: 29018045)
Eur J Immunol. 2008 Nov;38(11):3219-25. (PMID: 18991284)
J Med Genet. 2010 Sep;47(9):595-600. (PMID: 20798128)
Immunity. 2019 May 21;50(5):1132-1148. (PMID: 31117010)
J Exp Med. 1980 Apr 1;151(4):853-62. (PMID: 6966310)
Nat Rev Immunol. 2006 Dec;6(12):919-29. (PMID: 17124513)
Eur J Immunol. 2014 Feb;44(2):573-84. (PMID: 24227526)
Nat Rev Immunol. 2012 Feb 17;12(3):180-90. (PMID: 22343569)
Blood. 2013 Jan 24;121(4):595-603. (PMID: 23160464)
Curr Opin Immunol. 2012 Jun;24(3):297-302. (PMID: 22341735)
Nat Methods. 2016 Sep;13(9):731-40. (PMID: 27348712)
Cell. 2000 Sep 1;102(5):565-75. (PMID: 11007475)
Proc Natl Acad Sci U S A. 1998 Dec 22;95(26):15781-6. (PMID: 9861047)
Nat Rev Immunol. 2008 Jun;8(6):421-34. (PMID: 18483500)
Anal Chem. 2003 Apr 15;75(8):1895-904. (PMID: 12713048)
Eur J Immunol. 2019 Apr;49(4):626-637. (PMID: 30636035)
J Immunol. 2006 Oct 15;177(8):5317-27. (PMID: 17015717)
J Exp Med. 1993 Nov 1;178(5):1473-81. (PMID: 8228801)
Nat Genet. 2000 Jun;25(2):173-6. (PMID: 10835631)
Br J Haematol. 2008 Jul;142(3):469-79. (PMID: 18547321)
Nat Rev Immunol. 2015 Mar;15(3):185-9. (PMID: 25677493)
J Immunol. 2008 Sep 15;181(6):3994-4003. (PMID: 18768854)
Traffic. 2011 Jun;12(6):762-73. (PMID: 21388490)
Eur J Immunol. 2021 Feb;51(2):408-413. (PMID: 32996581)
Nat Rev Mol Cell Biol. 2001 Feb;2(2):98-106. (PMID: 11252968)
J Immunol. 2015 Jul 15;195(2):564-75. (PMID: 26056253)
Cell Mol Life Sci. 2017 Feb;74(3):399-408. (PMID: 27585956)
J Med Genet. 2006 Apr;43(4):e14. (PMID: 16582076)
Nat Commun. 2018 Nov 28;9(1):5037. (PMID: 30487586)
معلومات مُعتمدة: 1160/IMPATH German Research Foundation; 01 EO 0803 German Federal Ministry of Education and Research; Saarland University; 2021/A2-Fol University of Freiburg
المشرفين على المادة: 0 (Qa-SNARE Proteins)
0 (STX11 protein, human)
تواريخ الأحداث: Date Created: 20240509 Date Completed: 20240509 Latest Revision: 20240605
رمز التحديث: 20240606
مُعرف محوري في PubMed: PMC11082451
DOI: 10.1084/jem.20221122
PMID: 38722309
قاعدة البيانات: MEDLINE
الوصف
تدمد:1540-9538
DOI:10.1084/jem.20221122