Results 71 to 80 of about 27,174 (202)

The Transcription Factor EGR2 Plays a Central Role in the Expansion and Function of TCRαβ + CD4 − CD8 − Double Negative T Cells in lpr Lupus Mice

Immunology, Volume 179, Issue 2, Page 224-235, October 2026.
The highly expanded TCRβ+ DNT cells in autoimmune‐prone B6/lpr mice are phenotypically and functionally different from the TCRβ+DNT cells of normal B6 mice. Conditional Egr2 deletion in B6/lpr mice not only reduces TCRβ+ DNT cell numbers but also tends to correct the phenotypic and functional abnormalities of DNT cells in B6/lpr mice. In addition, Egr2
Rujuan Dai, Zhuang Wang, Bettina Heid, Christopher M. Reilly, S. Ansar Ahmed   +4 more
wiley   +1 more source

Sarcoidosis and Autoinflammation

Inflammation and Regeneration, 2011
Sporadic early-onset sarcoidosis (EOS) and familial Blau syndrome (BS) form a distinct set of autoinflammatory diseases, both of which onset in infancy and show a clinical triad of dermatitis, arthritis and uveitis histologically composed of noncaseating epithelioid cell granuloma.
openaire   +2 more sources

A Multifaceted Interplay Among Hemophagocytosis, Interleukin‐18, and Type I Interferon Distinguishes Still Disease From Other Autoinflammatory Diseases

Arthritis &Rheumatology, Volume 78, Issue 9, Page 1961-1974, September 2026.
Objective The unknown pathophysiology and the lack of specific features for systemic juvenile idiopathic arthritis and adult‐onset Still disease (collectively known as Still disease; SD) delay diagnosis and appropriate treatment. The goal of this study was to identify features and mechanisms that distinguish SD from other systemic autoinflammatory ...
Yvonne M. Mueller, Charlotte Girard, Harmen J. G. van de Werken, Stefan J. Erkeland, Dwin G. B. Grashof, Laurie Vaillant, Renske J. H. den Dekker, Iris van Zuijen, Sharon Veenbergen, Fanny Coffin, Eric Bindels, Yvan Jamilloux, Katerina Laskari, Vassili Soumelis, Bruno Fautrel, Cem Gabay, Peter D. Katsikis   +16 more
wiley   +1 more source

When to consider an inborn error of immunity: clues for physicians

Internal Medicine Journal, Volume 56, Issue 9, Page 1488-1499, September 2026.
Abstract The term inborn errors of immunity (IEIs) refers to the rapidly expanding group of genetic disorders causing dysregulation of the immune system. With improved genetic testing in recent years, the number of defined IEIs and their range of phenotypic presentations has grown vastly, with more than 550 IEIs now described.
Meera Thangarajah, Lucinda J. Berglund
wiley   +1 more source

LUBAC deficiency perturbs TLR3 signaling to cause immunodeficiency and autoinflammation [PDF]

, 2016
The linear ubiquitin chain assembly complex (LUBAC), consisting of SHANK-associated RH-domain–interacting protein (SHARPIN), heme-oxidized IRP2 ubiquitin ligase-1 (HOIL-1), and HOIL-1–interacting protein (HOIP), is a critical regulator of inflammation ...
Torsten Hartwig, Rieser, E, Endres, C, Peltzer, N, Lucia Taraborrelli, Johannes Lemke, Darding, M, Lemke, J, Draber, P, Ren, H, Taraborrelli, L, Cornelia Endres, Michael Bergmann, Ferguson, BJ, Peter Draber, Brian J. Ferguson, Balazs Dome, Nieves Peltzer, Kovacs, I, Dome, B, Zinngrebe, J, Maurice Darding, Henning Walczak, Hartwig, T, Walczak, H, Julia Zinngrebe, Eva Rieser, Hongwei Ren, Silvia von Karstedt, von Karstedt, S, Ildikó Kovács, Bergmann, M   +31 more
core   +2 more sources

Clinical presentation, diagnosis, and treatment of chronic granulomatous disease

Frontiers in Pediatrics
Chronic granulomatous disease (CGD) is caused by an impaired respiratory burst reaction in phagocytes. CGD is an X-linked (XL) (caused by pathogenic variants in CYBB) or autosomal recessive inborn error of immunity (caused by pathogenic variants in CYBA,
Olga Staudacher, Horst von Bernuth, Horst von Bernuth, Horst von Bernuth, Horst von Bernuth   +4 more
doaj   +1 more source

The Src‐Family Kinases Hck, Fgr and Lyn Drive Immune Complex‐Mediated Glomerulonephritis Through the Syk Tyrosine Kinase

The FASEB Journal, Volume 40, Issue 16, 31 August 2026.
The study aimed to test the role of three Src‐family kinases, Hck, Fgr, and Lyn in immune complex glomerulonephritis. Hck, Fgr, and Lyn were overexpressed in human lupus nephritis and mouse nephrotoxic nephritis. Combined genetic deficiency of the three kinases practically completely protected mice from nephrotoxic nephritis.
Lukács S. Lesinszki, Dorottya Deli, Lili K. Boldizsár, Áron Pánczél, Attila Fintha, Michael G. Robson, Attila Mócsai   +6 more
wiley   +1 more source

Exploratory Analysis of the Inhibitory Effects of Propranolol on NLRP3 and Pyrin Inflammasomes

ACR Open Rheumatology, Volume 8, Issue 8, August 2026.
Objective Propranolol, a nonselective beta receptor blocking agents, impacts cAMP levels and is commonly used to treat hypertension and hemangioma in children and adults. Although there are reports indicating its anti‐inflammatory properties, the exact mechanism is not fully understood. Methods Murine and human monocytes and macrophages were exposed to
Renske J. de Jong, Christopher T. Neullens, Aljoscha Swoboda, Melanie Saers, Philipp Berger, Sudheendra Hebbar Subramanyam, Anastasia Wiener, Kim Ohl, Ralf Weiskirchen, Heidi Noels, Tilmann Kallinich, Julia Guthrie, Christoph Kessel, Klaus Tenbrock   +13 more
wiley   +1 more source

Human neutrophils drive skin autoinflammation by releasing interleukin (IL)-26

Autoinflammation is a sterile inflammatory process resulting from increased neutrophil infiltration and overexpression of IL-1 cytokines. The factors that trigger these events are, however, poorly understood. By investigating pustular forms of psoriasis,
Yatim, Ahmad, Grizzetti, Lorenzo, Baldo, Alessia, Gilliet, Michel, Vandenberghe-Durr, Sophie, Kuonen, François, Di Domizio, Jeremy, Jenelten, Raphael, Fries, Anissa, Paul, Carle, Conrad, Curdin, Modlin, Robert L   +11 more
core   +1 more source

Alzheimer's Protection by PLCγ2 Compacts Plaques, Redistributes Microglia, and Protects Synapses in AppNL‐G‐F Mice

Glia, Volume 74, Issue 8, August 2026.
The Alzheimer's disease protective PLCG2 P522R variant protects against amyloid‐induced synapse loss independently of amyloid load, promotes increased plaque compaction, and is associated with enhanced microglial engagement with diffuse amyloid. ABSTRACT The Alzheimer's disease protective P522R PLCG2 coding variant (rs72824905) is downstream of TREM2 ...
Ryan J. Bevan, Emily Maguire, Eilish Mackinnon, Elisa Salis, Thomas Phillips, Elena Simonazzi, Marieta Vassileva, Nicholas D. Allen, Julie Williams, Philip R. Taylor   +9 more
wiley   +1 more source