Results 1 to 10 of about 291 (97)
The Golden Card of Interleukin-1 Blockers in Systemic Inflammasomopathies of Childhood [PDF]
International Journal of Molecular SciencesA growing number of systemic hereditary inflammatory diseases characterized by periodic fevers and elevated acute-phase proteins during flares has been linked to deregulated inflammasome function and excessive bioactivity of interleukin (IL)-1. All these conditions respond, at varying degrees, to the specific blockade of IL-1.
Donato Rigante, Rigante Donato
exaly +5 more sources
Pathogenic insights from genetic causes of autoinflammatory inflammasomopathies and interferonopathies [PDF]
Journal of Allergy and Clinical Immunology, 2022 A number of systemic autoinflammatory diseases arise from gain-of-function mutations in genes encoding IL-1-activating inflammasomes or cytoplasmic nucleic acid sensors including the receptor and sensor STING and result in increased IL-1 and type I interferon production, respectively.
Bin Lin, Raphaela Goldbach-Mansky
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Zdorovʹe RebenkaInflammasomopathies and other enhanced interleukin (IL)-1 signaling syndromes represent a significant cluster of systemic autoinflammatory diseases (SAIDs) which are monogenic disorders characterized by episodic spontaneous multisystemic inflammation ...
O.V. Shvaratska +7 more
exaly +4 more sources
Zdorovʹe RebenkaSystemic autoinflammatory diseases (SAIDs) are considered dysregulation disorders of the innate immune system characterized by systemic sterile inflammation independent of infection and autoreactive antibodies or antigen-specific T cells ...
О.В. Шварацька +7 more
exaly +4 more sources
NLRC4 inflammasomopathies [PDF]
Current Opinion in Allergy and Clinical Immunology, 2017 Purpose of reviewThe purpose of the review is to highlight developments in autoinflammatory diseases associated with gain-of-function mutations in the gene encoding NLR-family CARD-containing protein 4 (NLRC4), the NLRC4-inflammasomopathies.Recent findingsThree years since the identification of the first autoinflammation with infantile enterocolitis ...
Neil Romberg +2 more
exaly +10 more sources
Indian Journal of Rheumatology, 2022 Inherited disorders of the inflammasome pathway causes dysregulated inflammasome activation, which presents with inflammation and other clinical features linked to the defective protein.
Nayan Patel Sureja, Liza Rajasekhar
doaj +2 more sources
Molecular biology of autoinflammatory diseases [PDF]
Inflammation and Regeneration, 2021 The long battle between humans and various physical, chemical, and biological insults that cause cell injury (e.g., products of tissue damage, metabolites, and/or infections) have led to the evolution of various adaptive responses.
Junya Masumoto +7 more
doaj +2 more sources
The autoinflammation-associated NLRC4V341A mutation increases microbiota-independent IL-18 production but does not recapitulate human autoinflammatory symptoms in mice [PDF]
Frontiers in Immunology, 2023 BackgroundAutoinflammation with infantile enterocolitis (AIFEC) is an often fatal disease caused by gain-of-function mutations in the NLRC4 inflammasome.
Elien Eeckhout +19 more
doaj +2 more sources
Crosstalk between Interleukin-1β and Type I Interferons Signaling in Autoinflammatory Diseases [PDF]
Cells, 2021 Interleukin-1β (IL-1β) and type I interferons (IFNs) are major cytokines involved in autoinflammatory/autoimmune diseases. Separately, the overproduction of each of these cytokines is well described and constitutes the hallmark of inflammasomopathies and
Philippe Georgel
doaj +2 more sources
Divergence of IL-1, IL-18, and cell death in NLRP3 inflammasomopathies [PDF]
Journal of Clinical Investigation, 2013 The inflammasome is a cytoplasmic multiprotein complex that promotes proinflammatory cytokine maturation in response to host- and pathogen-derived signals. Missense mutations in cryopyrin (NLRP3) result in a hyperactive inflammasome that drives overproduction of the proinflammatory cytokines IL-1β and IL-18, leading to the cryopyrin-associated periodic
Lori Broderick +2 more
exaly +3 more sources