ABOUT THE RESEARCHER

OVERVIEW

Dr. Janssen’s research focuses on the molecular mechanisms underlying autoimmunity and primary immunodeficiencies.

PUBLICATIONS

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  1. Pulmonary manifestations of immune dysregulation in CTLA-4 haploinsufficiency and LRBA deficiency. Pediatr Pulmonol. 2021 Mar 12. View abstract
  2. Macabre TH2 skewing in DOCK8 deficiency. J Allergy Clin Immunol. 2021 Mar 02. View abstract
  3. DOCK8 Expression in Regulatory T Cells Maintains their Stability and Limits Contact Hypersensitivity. J Invest Dermatol. 2020 Nov 07. View abstract
  4. Distinct clinical and immunological features of SARS-CoV-2-induced multisystem inflammatory syndrome in children. J Clin Invest. 2020 11 02; 130(11):5942-5950. View abstract
  5. DOCK8 is essential for LFA-1-dependent positioning of T follicular helper cells in germinal centers. JCI Insight. 2020 08 06; 5(15). View abstract
  6. Th17 reprogramming of T cells in systemic juvenile idiopathic arthritis. JCI Insight. 2020 03 26; 5(6). View abstract
  7. Cytoskeletal tension actively sustains the migratory T-cell synaptic contact. EMBO J. 2020 03 02; 39(5):e102783. View abstract
  8. The immunologic features of patients with early-onset and polyautoimmunity. Clin Immunol. 2020 02; 211:108326. View abstract
  9. Defining a new immune deficiency syndrome: MAN2B2-CDG. J Allergy Clin Immunol. 2020 03; 145(3):1008-1011. View abstract
  10. Primary immunodeficiencies caused by mutations in actin regulatory proteins. Immunol Rev. 2019 01; 287(1):121-134. View abstract
  11. WASP-mediated regulation of anti-inflammatory macrophages is IL-10 dependent and is critical for intestinal homeostasis. Nat Commun. 2018 05 03; 9(1):1779. View abstract
  12. DOCK8 enforces immunological tolerance by promoting IL-2 signaling and immune synapse formation in Tregs. JCI Insight. 2017 10 05; 2(19). View abstract
  13. Deficiency of base excision repair enzyme NEIL3 drives increased predisposition to autoimmunity. J Clin Invest. 2016 11 01; 126(11):4219-4236. View abstract
  14. A DOCK8-WIP-WASp complex links T cell receptors to the actin cytoskeleton. J Clin Invest. 2016 10 03; 126(10):3837-3851. View abstract
  15. Heterozygosity for transmembrane activator and calcium modulator ligand interactor A144E causes haploinsufficiency and pneumococcal susceptibility in mice. J Allergy Clin Immunol. 2017 Apr; 139(4):1293-1301.e4. View abstract
  16. Next-Generation Sequencing Reveals Restriction and Clonotypic Expansion of Treg Cells in Juvenile Idiopathic Arthritis. Arthritis Rheumatol. 2016 07; 68(7):1758-68. View abstract
  17. Regulatory T-cell deficiency and immune dysregulation, polyendocrinopathy, enteropathy, X-linked-like disorder caused by loss-of-function mutations in LRBA. J Allergy Clin Immunol. 2015 Jan; 135(1):217-27. View abstract
  18. Dedicator of cytokinesis 8-deficient patients have a breakdown in peripheral B-cell tolerance and defective regulatory T cells. J Allergy Clin Immunol. 2014 Dec; 134(6):1365-1374. View abstract
  19. Flow cytometry diagnosis of dedicator of cytokinesis 8 (DOCK8) deficiency. J Allergy Clin Immunol. 2014 Jul; 134(1):221-3. View abstract
  20. A119: deep sequencing analysis of the T regulatory and T effector repertoire in juvenile idiopathic arthritis. Arthritis Rheumatol. 2014 Mar; 66 Suppl 11:S156. View abstract
  21. TRIF signaling is essential for TLR4-driven IgE class switching. J Immunol. 2014 Mar 15; 192(6):2651-8. View abstract
  22. Flow cytometry biomarkers distinguish DOCK8 deficiency from severe atopic dermatitis. Clin Immunol. 2014 Feb; 150(2):220-4. View abstract
  23. Dominant gain-of-function STAT1 mutations in FOXP3 wild-type immune dysregulation-polyendocrinopathy-enteropathy-X-linked-like syndrome. J Allergy Clin Immunol. 2013 Jun; 131(6):1611-23. View abstract
  24. Epicutaneous sensitization results in IgE-dependent intestinal mast cell expansion and food-induced anaphylaxis. J Allergy Clin Immunol. 2013 Feb; 131(2):451-60.e1-6. View abstract
  25. DOCK8 functions as an adaptor that links TLR-MyD88 signaling to B cell activation. Nat Immunol. 2012 May 13; 13(6):612-20. View abstract
  26. Anakinra as first-line disease-modifying therapy in systemic juvenile idiopathic arthritis: report of forty-six patients from an international multicenter series. Arthritis Rheum. 2011 Feb; 63(2):545-55. View abstract
  27. Adaptor protein function in lymphocyte development and activation: an analysis of transmembrane adaptors deficient in PLC-gamma binding. 2004. View abstract
  28. Linker for activation of B cells: a functional equivalent of a mutant linker for activation of T cells deficient in phospholipase C-gamma1 binding. J Immunol. 2004 Jun 01; 172(11):6810-9. View abstract
  29. The importance of three membrane-distal tyrosines in the adaptor protein NTAL/LAB. J Biol Chem. 2004 Mar 19; 279(12):11229-35. View abstract
  30. Adaptor proteins in lymphocyte activation. Curr Opin Immunol. 2003 Jun; 15(3):269-76. View abstract
  31. LAB: a new membrane-associated adaptor molecule in B cell activation. Nat Immunol. 2003 Feb; 4(2):117-23. View abstract
  32. Minimal requirement of tyrosine residues of linker for activation of T cells in TCR signaling and thymocyte development. J Immunol. 2003 Jan 01; 170(1):325-33. View abstract
  33. Molecular cloning of a novel gene encoding a membrane-associated adaptor protein (LAX) in lymphocyte signaling. J Biol Chem. 2002 Nov 29; 277(48):46151-8. View abstract
  34. Pleiotropic defects in TCR signaling in a Vav-1-null Jurkat T-cell line. EMBO J. 2002 Sep 16; 21(18):4809-19. View abstract