Human DEF6 deficiency underlies an immunodeficiency syndrome with systemic autoimmunity and aberrant CTLA-4 homeostasis

Autor: Nina K. Serwas, Birgit Hoeger, Rico C. Ardy, Sigrun V. Stulz, Zhenhua Sui, Nima Memaran, Marie Meeths, Ana Krolo, Özlem Yüce Petronczki, Laurène Pfajfer, Tie Z. Hou, Neil Halliday, Elisangela Santos-Valente, Artem Kalinichenko, Alan Kennedy, Emily M. Mace, Malini Mukherjee, Bianca Tesi, Anna Schrempf, Winfried F. Pickl, Joanna I. Loizou, Renate Kain, Bettina Bidmon-Fliegenschnee, Jean-Nicolas Schickel, Salomé Glauzy, Jakob Huemer, Wojciech Garncarz, Elisabeth Salzer, Iro Pierides, Ivan Bilic, Jens Thiel, Peter Priftakis, Pinaki P. Banerjee, Elisabeth Förster-Waldl, David Medgyesi, Wolf-Dietrich Huber, Jordan S. Orange, Eric Meffre, David M. Sansom, Yenan T. Bryceson, Amnon Altman, Kaan Boztug

Publikováno v: Nature Communications, Vol 10, Iss 1, Pp 1-15 (2019)

CTLA-4 is critical for balancing protective immunity with self-tolerance. Here the authors identify homozygous DEF6 mutations in patients with severe autoimmunity, one of which received and responds to CTLA-4-Ig, and show that DEF6 is crucial for CTL

Externí odkaz: https://doaj.org/article/2b28c1d1ae924d86a02c99db5aac2d80

CXCR4-related increase of circulating human lymphoid progenitors after allogeneic hematopoietic stem cell transplantation.

Autor: Salomé Glauzy, Isabelle André-Schmutz, Jérôme Larghero, Sophie Ezine, Régis Peffault de Latour, Hélène Moins-Teisserenc, Sophie Servais, Marie Robin, Gérard Socié, Emmanuel Clave, Antoine Toubert

Publikováno v: PLoS ONE, Vol 9, Iss 3, p e91492 (2014)

Immune recovery after profound lymphopenia is a major challenge in many clinical situations, such as allogeneic hematopoietic stem cell transplantation (allo-HSCT). Recovery depends, in a first step, on hematopoietic lymphoid progenitors production i

Externí odkaz: https://doaj.org/article/70eac6067aef47979fdab7b3122e8929

Regulatory T Cell Stability and Migration Are Dependent on mTOR

Autor: Bruno Lucas, Ivan Nemazanyy, Sylvie Grégoire, Benoît L. Salomon, Emilie Ronin, Aurélie Durand, Salomé Glauzy, Martina Lubrano di Ricco, Jordane Divoux, Romain Vallion, Delphine Fradin, Yannis Lombardi

Publikováno v: Journal of Immunology
Journal of Immunology, Publisher : Baltimore : Williams & Wilkins, c1950-. Latest Publisher : Bethesda, MD : American Association of Immunologists, 2020, 205 (7), pp.1799-1809. ⟨10.4049/jimmunol.1901480⟩
Journal of Immunology, 2020, 205 (7), pp.1799-1809. ⟨10.4049/jimmunol.1901480⟩

CD4+ Foxp3+ regulatory T cells (Treg) are essential to maintain immune tolerance, as their loss leads to a fatal autoimmune syndrome in mice and humans. Conflicting findings have been reported concerning their metabolism. Some reports found that Treg

Externí odkaz: https://explore.openaire.eu/search/publication?articleId=doi_dedup___::53d9267abb3e3f2c64dc465af34893a4
https://pubmed.ncbi.nlm.nih.gov/32839235

Brief Report: Defective Early B Cell Tolerance Checkpoints in Sjögren's Syndrome Patients

Autor: Jacques-Eric Gottenberg, Joel Sng, Anne-Sophie Korganow, Jason M. Bannock, David Saadoun, Salomé Glauzy, Eric Meffre, Patrice Cacoub

Publikováno v: Arthritis & Rheumatology. 69:2203-2208

Objective: Central and peripheral B cell tolerance checkpoints are defective in many patients with autoimmune diseases, but the functionality of each discrete checkpoint has not been assessed in SS patients. Methods: Using a PCR-based approach that a

Externí odkaz: https://explore.openaire.eu/search/publication?articleId=doi_________::822d95379a7a60a648471607ab554ed2
https://doi.org/10.1002/art.40215

Self-reactive VH4-34–expressing IgG B cells recognize commensal bacteria

Autor: Jean-Laurent Casanova, Capucine Picard, Christopher Massad, Nicolas Chamberlain, Isabelle Isnardi, Steven M. Holland, Gulbu Uzel, Yen Shing Ng, Nathan Katz, Salomé Glauzy, Eric Meffre, Anne Puel, Jean-Nicolas Schickel

Publikováno v: The Journal of Experimental Medicine

The human VH4-34 gene segment encodes intrinsically self-reactive antibodies that recognize I/i carbohydrates. Schickel et al. show that these self-reactive clones may represent an innate-like B cell population specialized in the containment of comme

Externí odkaz: https://explore.openaire.eu/search/publication?articleId=doi_dedup___::c13ae487e110826d3bd967a0410b1091
https://doi.org/10.1084/jem.20160201