Pyrin

A tripartite motif protein that consists of an N-terminal pyrin domain, a central coiled-coil region and B-box type ZINC FINGER, and C-terminal regions that mediate homotrimerization and interactions with other proteins (the B30.2/SPRY DOMAIN). It is expressed primarily by mature GRANULOCYTES and associates with the cytoskeleton in the perinuclear area as well as AUTOPHAGOSOMES, where it co-ordinates the assembly of AUTOPHAGY-RELATED PROTEINS and degradation of INFLAMMASOME components. It functions in INNATE IMMUNITY and INFLAMMATION; mutations in the Pyrin protein (MEFV) gene are associated with FAMILIAL MEDITERRANEAN FEVER.

Also Known As:

Networked: 409 relevant articles (13 outcomes, 31 trials/studies)

Relationship Network

Bio-Agent Context: Research Results

Experts

1.	Kastner, Daniel L: 15 articles (01/2022 - 12/2002)
2.	Chae, Jae Jin: 14 articles (01/2022 - 12/2002)
3.	Aksentijevich, Ivona: 11 articles (01/2022 - 12/2002)
4.	Masters, Seth L: 11 articles (01/2022 - 01/2006)
5.	Balci-Peynircioglu, Banu: 8 articles (01/2022 - 09/2008)
6.	Ozen, Seza: 7 articles (01/2022 - 09/2003)
7.	McDermott, Michael F: 7 articles (01/2017 - 12/2002)
8.	Aksentijevich, I: 6 articles (10/2011 - 06/2000)
9.	Bliska, James B: 5 articles (10/2021 - 01/2019)
10.	Kanneganti, Thirumala-Devi: 5 articles (01/2021 - 02/2017)

Related Diseases

1.	Infections 01/01/2019 - "In healthy carriers of FMF mutations who have the subclinical inflammatory phenotype, the increased activity of pyrin might have been protective against endemic infections over human history. " 01/01/2020 - "Recent results from infection studies with human phagocytes and mice producing pyrin B30.2 FMF variants show that gain of function MEFV mutations bypass inhibition by YopM. " 01/01/2020 - "Insights into pyrin regulation have come from studies of several Yersinia effectors, which are injected into phagocytes and interact with the RhoA-PRK-pyrin axis during infection. " 01/01/2021 - "Here we found that AIM2 regulates the innate immune sensors pyrin and ZBP1 to drive inflammatory signalling and a form of inflammatory cell death known as PANoptosis, and provide host protection during infections with herpes simplex virus 1 and Francisella novicida. " 01/01/2020 - "We propose that in humans, Pyrin may have evolved to fight highly pathogenic infections."
2.	Skin Diseases (Skin Disease) 03/30/2016 - "Successful therapy targeting IL-1β has been initiated in one patient, resolving pyrin-associated autoinflammation with neutrophilic dermatosis. " 01/01/2022 - "In addition, distinct mutations in MEFV, the gene encoding pyrin, cause a different rare autoinflammatory disease termed pyrin-associated autoinflammation with neutrophilic dermatosis (PAAND). " 12/01/2021 - "Pyrin-associated autoinflammation with neutrophilic dermatosis (PAAND) is a monogenic autoinflammatory disorder with autosomal dominant inheritance and has been associated with monoallelic p.Ser242Arg and p.Glu244Lys variations in the MEFV gene. " 12/01/2021 - "Homozygous MEFV Gene Variant and Pyrin-Associated Autoinflammation With Neutrophilic Dermatosis: A Family With a Novel Autosomal Recessive Mode of Inheritance." 11/03/2021 - "Phenotypic analysis of pyrin-associated autoinflammation with neutrophilic dermatosis patients during treatment."
3.	Inflammation (Inflammations) 12/08/2021 - "The present study investigated the role of pyrin/TRIM20 in inflammation and the pathogenesis of FMF. " 01/01/2021 - "In the past year, a number of studies have addressed the contributing factors to the inflammation caused by the mutations in pyrin; this has included epigenetic studies as well. " 01/01/2007 - "This study confirmed that reduced expression of the MEFV gene is associated with inflammation and that it may be one of the pathogenic mechanisms of the attacks of inflammation in FMF patients, along with disease-associated variations in pyrin." 01/01/2022 - "It encodes pyrin, which controls inflammation. " 01/01/2021 - "Nucleotide‑binding oligomerization domain NOD‑like receptor containing pyrin domain 3 (NLRP3)‑mediated inflammation participates in the development of renal injury. "
4.	Fever (Fevers) 01/01/2023 - "Post COVID recurrent fever in children with polymorphisms in the innate immunity regulator, pyrin; MEFV gene." 01/01/2019 - "The Changing Concepts Regarding the Mediterranean Fever Gene: Toward a Spectrum of Pyrin-Associated Autoinflammatory Diseases with Variable Heredity." 01/01/2018 - "The Mediterranean fever (MEFV) gene is responsible for FMF and encodes pyrin that suppresses the inflammatory response. " 01/01/2016 - "MEFV (Mediterranean fever) gene encoding pyrin regulates inflammatory responses. " 02/06/2015 - "Genetic mutations in the Mediterranean fever (MEFV) gene, encoding pyrin, influence the severity of RA, but the underlying mechanisms are not completely understood. "
5.	Familial Mediterranean Fever (Periodic Disease) 10/03/2023 - "Understanding pyrin regulation is crucial due to its association with dysregulated inflammatory responses, including Familial Mediterranean Fever (FMF), linked to pyrin gene mutations. " 07/01/2023 - "Familial Mediterranean fever (FMF), caused by mutations in the pyrin-encoding MEFV gene, is characterized by uncontrolled caspase-1 activation and IL-1β secretion. " 01/01/2022 - "Familial Mediterranean fever (FMF) is an autoinflammatory disease caused by pathogenic variants of the MEFV gene, which encodes pyrin. " 12/10/2020 - "Familial Mediterranean fever (FMF) is caused by dysfunction of the MEFV gene product, pyrin. " 11/15/2020 - "Mutations in MEFV, the gene encoding pyrin in humans, are associated with the autoinflammatory disorder familial Mediterranean fever. "

Related Drugs and Biologics

1.	Inflammasomes
2.	Nucleotides
3.	Leucine (L-Leucine)
4.	Proteins (Proteins, Gene)
5.	NLR Proteins
6.	Dexmedetomidine
7.	Oleanolic Acid
8.	Apelin
9.	Caffeine (No Doz)
10.	Anti-Bacterial Agents (Antibiotics)

Related Therapies and Procedures

1.	Electroacupuncture
2.	Therapeutics
3.	Ligation
4.	Lasers (Laser)
5.	Subcutaneous Injections