NLRP3mutation and cochlear autoinflammation cause syndromic and nonsyndromic hearing loss DFNA34 responsive to anakinra therapy

NLRP3 Is Expressed in the Spiral Ganglion Neurons and Associated with Both Syndromic and Nonsyndromic Sensorineural Deafness

(2016) Penghui Chen et al.   NEURAL PLASTICITY

Myeloid lineage–restricted somatic mosaicism of NLRP3 mutations in patients with variant Schnitzler syndrome

(2015) Heleen D. de Koning et al.   JOURNAL OF ALLERGY AND CLINICAL IMMUNOLOGY

A small-molecule inhibitor of the NLRP3 inflammasome for the treatment of inflammatory diseases

(2015) Rebecca C Coll et al.   NATURE MEDICINE

NLRP3 deficiency protects from type 1 diabetes through the regulation of chemotaxis into the pancreatic islets

(2015) Changyun Hu et al.   PROCEEDINGS OF THE NATIONAL ACADEMY OF SCIENCES OF THE UNITED STATES OF AMERICA

Mechanism of NLRP3 inflammasome activation

(2014) Fayyaz S. Sutterwala et al.   Annals of the New York Academy of Sciences

IL-1 Blockade in Autoinflammatory Syndromes

(2014) Adriana A. Jesus et al.   Annual Review of Medicine

Early efficacy trial of anakinra in corticosteroid-resistant autoimmune inner ear disease

(2014) Andrea Vambutas et al.   JOURNAL OF CLINICAL INVESTIGATION

Tissue-resident macrophages originate from yolk-sac-derived erythro-myeloid progenitors

(2014) Elisa Gomez Perdiguero et al.   NATURE

Slc26a4-insufficiency causes fluctuating hearing loss and stria vascularis dysfunction

(2014) Taku Ito et al.   NEUROBIOLOGY OF DISEASE

SomaticNLRP3mosaicism in Muckle-Wells syndrome. A genetic mechanism shared by different phenotypes of cryopyrin-associated periodic syndromes

(2013) Kenji Nakagawa et al.   ANNALS OF THE RHEUMATIC DISEASES

Current concepts in age-related hearing loss: Epidemiology and mechanistic pathways

(2013) Tatsuya Yamasoba et al.   HEARING RESEARCH

Sustained response and prevention of damage progression in patients with neonatal-onset multisystem inflammatory disease treated with anakinra: A cohort study to determine three- and five-year outcomes

(2012) Cailin H. Sibley et al.   ARTHRITIS AND RHEUMATISM

Inflammasome Activators Induce Interleukin-1α Secretion via Distinct Pathways with Differential Requirement for the Protease Function of Caspase-1

(2012) Olaf Groß et al.   IMMUNITY

NLRP1-Dependent Pyroptosis Leads to Acute Lung Injury and Morbidity in Mice

(2012) M. Kovarova et al.   JOURNAL OF IMMUNOLOGY

The calcium-sensing receptor regulates the NLRP3 inflammasome through Ca2+ and cAMP

(2012) Geun-Shik Lee et al.   NATURE

Constitutively Activated NLRP3 Inflammasome Causes Inflammation and Abnormal Skeletal Development in Mice

(2012) Sheri L. Bonar et al.   PLoS One

Perivascular-resident macrophage-like melanocytes in the inner ear are essential for the integrity of the intrastrial fluid-blood barrier

(2012) W. Zhang et al.   PROCEEDINGS OF THE NATIONAL ACADEMY OF SCIENCES OF THE UNITED STATES OF AMERICA

High incidence of NLRP3 somatic mosaicism in patients with chronic infantile neurologic, cutaneous, articular syndrome: Results of an international multicenter collaborative study

(2011) Naoko Tanaka et al.   ARTHRITIS AND RHEUMATISM

The inflammasomes in health and disease: from genetics to molecular mechanisms of autoinflammation and beyond

(2011) Cristina Conforti-Andreoni et al.   Cellular & Molecular Immunology

Fatty acid and endotoxin activate inflammasomes in mouse hepatocytes that release danger signals to stimulate immune cells

(2011) Timea Csak et al.   HEPATOLOGY

Intracellular sensing of microbes and danger signals by the inflammasomes

(2011) Gabor L. Horvath et al.   IMMUNOLOGICAL REVIEWS

Differential Expression of NLRP3 among Hematopoietic Cells

(2011) G. Guarda et al.   JOURNAL OF IMMUNOLOGY

Cryopyrin-Associated Periodic Syndromes

(2011) Neda Ahmadi et al.   OTOLARYNGOLOGY-HEAD AND NECK SURGERY

A Noncoding Point Mutation of Zeb1 Causes Multiple Developmental Malformations and Obesity in Twirler Mice

(2011) Kiyoto Kurima et al.   PLoS Genetics

Cell Type–Specific Transcriptome Analysis Reveals a Major Role for Zeb1 and miR-200b in Mouse Inner Ear Morphogenesis

(2011) Ronna Hertzano et al.   PLoS Genetics

Efficacy and safety of anakinra therapy in pediatric and adult patients with the autoinflammatory Muckle-Wells syndrome

(2010) Jasmin B. Kuemmerle-Deschner et al.   ARTHRITIS AND RHEUMATISM

Resident macrophages in the cochlear blood-labyrinth barrier and their renewal via migration of bone-marrow-derived cells

(2010) Xiaorui Shi   CELL AND TISSUE RESEARCH

NLRP3 Plays a Critical Role in the Development of Experimental Autoimmune Encephalomyelitis by Mediating Th1 and Th17 Responses

(2010) D. Gris et al.   JOURNAL OF IMMUNOLOGY

A role for mitochondria in NLRP3 inflammasome activation

(2010) Rongbin Zhou et al.   NATURE

Horror Autoinflammaticus: The Molecular Pathophysiology of Autoinflammatory Disease

(2009) Seth L. Masters et al.   Annual Review of Immunology

Long-term efficacy of the interleukin-1 receptor antagonist anakinra in ten patients with neonatal-onset multisystem inflammatory disease/chronic infantile neurologic, cutaneous, articular syndrome

(2009) Bénédicte Neven et al.   ARTHRITIS AND RHEUMATISM

Inflammasome-Mediated Disease Animal Models Reveal Roles for Innate but Not Adaptive Immunity

(2009) Susannah D. Brydges et al.   IMMUNITY

A Mutation in the Nlrp3 Gene Causing Inflammasome Hyperactivation Potentiates Th17 Cell-Dominant Immune Responses

(2009) Guangxun Meng et al.   IMMUNITY

Brain multiple sclerosis-like lesions in a patient with Muckle-Wells syndrome

(2009) S. Compeyrot-Lacassagne et al.   RHEUMATOLOGY

The NALP3 inflammasome is involved in the innate immune response to amyloid-β

(2008) Annett Halle et al.   NATURE IMMUNOLOGY