PFAPA syndrome is linked to dysregulated IL-1β production (original) (raw)
IL-1β and caspase-1 drive autoinflammatory disease independently of IL-1α or caspase-8 in a mouse model of Familial Mediterranean Fever
Bhesh Raj Sharma
Journal of Immunology, 2017
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Periodic fever, aphthous stomatitis, pharyngitis, and adenitis (PFAPA) is a disorder of innate immunity and Th1 activation responsive to IL-1 blockade
Henry Feder
Proceedings of the National Academy of Sciences, 2011
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Autoinflammation: The prominent role of IL-1 in monogenic autoinflammatory diseases and implications for common illnesses
Raphaela Goldbach-mansky
Journal of Allergy and Clinical Immunology, 2009
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Undifferentiated recurrent fevers in pediatrics are clinically distinct from PFAPA syndrome but retain an IL-1 signature
Marcella Bothwell
Clinical Immunology
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IL-Converting Enzyme/Caspase-1 Inhibitor VX-765 Blocks the Hypersensitive Response to an Inflammatory Stimulus in Monocytes from Familial Cold Autoinflammatory Syndrome Patients
John Randle
The Journal of Immunology, 2005
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Dysregulated production of interleukin-1β upon activation of the NLRP3 inflammasome in patients with familial Mediterranean fever
Despoina Vassou
Human Immunology, 2015
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Profile of blood cells and inflammatory mediators in periodic fever, aphthous stomatitis, pharyngitis and adenitis (PFAPA) syndrome
Per Wekell
BMC Pediatrics, 2010
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The 423Q polymorphism of the X-linked inhibitor of apoptosis gene influences monocyte function and is associated with periodic fever
Maurizia Baldi
Arthritis & Rheumatism, 2009
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Increased NLRP3-dependent interleukin 1 secretion in patients with familial Mediterranean fever: correlation with MEFV genotype
Marco Gattorno
Annals of the Rheumatic Diseases, 2014
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Primer: inflammasomes and interleukin 1β in inflammatory disorders
Michael F . McDermott
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Activated phenotype of circulating neutrophils in familial Mediterranean fever
Martin Petrek, Gayane Manukyan
Immunobiology, 2013
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Increased Intracellular Oxygen Radical Production in Neutrophils During Febrile Episodes of Periodic Fever, Aphthous Stomatitis, Pharyngitis, and Cervical Adenitis Syndrome
Anna Karlsson, Per Wekell
Arthritis & Rheumatism, 2013
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The emerging role of interleukin-1β in autoinflammatory diseases
Helen Lachmann, Thirusha Lane
Arthritis & Rheumatism, 2011
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Dysregulated mature IL-1 production in familial Mediterranean fever
M. Yasunami
Rheumatology, 2014
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Secretory Activity of Neutrophils Correlates With Genotype in Familial Mediterranean Fever
Horst Von Bernuth
Arthritis & rheumatology (Hoboken, N.J.), 2016
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Cytosolic, Autocrine Alpha-1 Proteinase Inhibitor (A1PI) Inhibits Caspase-1 and Blocks IL-1β Dependent Cytokine Release in Monocytes
Farshid Rouhani
PLoS ONE, 2012
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The B30.2 domain of pyrin, the familial Mediterranean fever protein, interacts directly with caspase-1 to modulate IL-1β production
Jae Jin Chae
Proceedings of the National Academy of Sciences, 2006
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Engaging anti-inflammatory mechanisms and triggering inflammatory effector apoptosis during Familial Mediterranean Fever attack
Tigran Davtyan
Inflammation …, 2008
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The Familial Mediterranean Fever Gene as a Modifier of Periodic Fever, Aphthous Stomatitis, Pharyngitis, and Adenopathy Syndrome
Shai Padeh
Seminars in Arthritis and Rheumatism, 2011
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Human Procaspase-1 Variants with Decreased Enzymatic Activity Are Associated with Febrile Episodes and May Contribute to Inflammation via RIP2 and NF- B Signaling
Engin Yilmaz
The Journal of Immunology, 2014
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Response to IL-1-Receptor Antagonist in a Child with Familial Cold Autoinflammatory Syndrome
Grainne O'Regan
Pediatric Dermatology, 2007
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Ex vivo PBMC cytokine profile in familial Mediterranean fever patients: Involvement of IL-1β, IL-1α and Th17-associated cytokines and decrease of Th1 and Th2 cytokines
Jean-claude Lecron
Cytokine, 2014
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Immune Dysregulation in the Tonsillar Microenvironment of Periodic Fever, Aphthous Stomatitis, Pharyngitis, Adenitis (PFAPA) Syndrome
Marcella Bothwell
Journal of Clinical Immunology, 2019
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The familial Mediterranean fever protein, pyrin, is cleaved by caspase-1 and activates NF-κB through its N-terminal fragment
Jae Jin Chae
Blood, 2008
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Horror Autoinflammaticus : The Molecular Pathophysiology of Autoinflammatory Disease *
Anna Simon
Annual Review of Immunology, 2009
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Interleukin 1 inhibitors in monogenic autoinflammatory diseases – one size does not fit all
Ewa Wiesik-szewczyk
Reumatologia/Rheumatology, 2021
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Differential activation of the inflammasome by caspase-1 adaptors ASC and Ipaf
Wyne Lee
Nature, 2004
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Inflammation in Mice Ectopically Expressing Human PAPA Syndrome-Associated PSTPIP1 A230T Mutant Proteins
Donghai Wang
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Activation of the NLRP3 Inflammasome and Elevation of Interleukin-1β Secretion in Infection by Sever Fever with Thrombocytopenia Syndrome Virus
Zheng Xing
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Interleukin-1 as a Key Factor in the Development of Inflammatory Diseases
Reza Shiari
Archives of Pediatric Infectious Diseases, 2014
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