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   Clin Exp Rheumatol. 2002 Jul-Aug;20(4 Suppl 26):S45-53.
Fire and ICE: the role of pyrin domain-containing proteins in inflammation
and apoptosis.
   Gumucio DL, Diaz A, Schaner P, Richards N, Babcock C, Schaller M,
   Cesena T.
   Department of Cell and Developmental Biology, University of Michigan,
   Ann Arbor 48109-0616, USA. dgumucio@umich.edu
   The genetic bases for several human autoinflammatory syndromes have
   recently been identified, and the mutated proteins responsible for
   these diseases are rapidly being characterized. Here, we examine two
   of these newly identified proteins, pyrin (also called marenostrin,
   product of the familial Mediterranean fever locus, MEFV) and cryopyrin
   (product of the CAIS1 locus, and mutated in familial cold urticaria,
   Muckle Wells syndrome and chronic infantile neurological cutaneous and
   articular syndrome). Both pyrin and cryopyrin contain an N-terminal
   domain that encodes a death domain-related structure, now known as the
   pyrin domain, or PyD. We trace the molecular interactions mediated by
   these PyDs, examine the evolution of the family of molecules
   containing this domain, and discuss the function of PyD-containing
   proteins and their homologues. Synthesis of the available data
   indicates that both pyrin and cryopyrin interact via their PyDs with a
   common adaptor protein, ASC. ASC itself participates in at least three
   important cellular processes: apoptosis, recruitment and activation of
   pro-caspase-1 (with associated processing and secretion of IL-1beta),
   and activation of NF-kappaB (a transcription factor involved in both
   initiation and resolution of the inflammatory response). Through
   PyD:PyD interactions, pyrin and cryopyrin, as well as several related,
   but still uncharacterized PyD containing proteins, appear to modulate
   the activity of all three of these processes, each of which plays a
   crucial role in the inflammatory pathways that characterize the innate
   immune system.
   PMID: 12371636 [PubMed - indexed for MEDLINE]
Publication Types, MeSH Terms, Substances, Grant Support
Publication Types:
     * Research Support, Non-U.S. Gov't
     * Research Support, U.S. Gov't, P.H.S.
     * Review
MeSH Terms:
     * Apoptosis/physiology*
     * Autoimmune Diseases/genetics*
     * Autoimmune Diseases/immunology
     * Autoimmune Diseases/physiopathology
     * Blood Proteins/chemistry
     * Blood Proteins/genetics
     * Blood Proteins/physiology*
     * Carrier Proteins/chemistry
     * Carrier Proteins/genetics
     * Carrier Proteins/physiology*
     * Cytoskeletal Proteins
     * Familial Mediterranean Fever/genetics
     * Familial Mediterranean Fever/physiopathology
     * Humans
     * Inflammation/physiopathology*
     * Proteins/chemistry
     * Proteins/genetics
     * Proteins/physiology*
Substances:
     * Blood Proteins
     * Carrier Proteins
     * Cytoskeletal Proteins
     * NLRP3 protein, human
     * Proteins
     * marenostrin
Grant Support:
     * K08 AI50092/AI/NIAID NIH HHS/United States
LinkOut - more resources
Other Literature Sources:
     * COS Scholar Universe
Medical:
     * Muckle-Wells Syndrome - Genetic Alliance
     * Muckle-Wells syndrome - Genetics Home Reference
     * familial cold autoinflammatory syndrome - Genetics Home Reference
     * Autoimmune Diseases - MedlinePlus Health Information
Supplemental Content
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       Curr Opin Rheumatol. 2003 Jan; 15(1):61-9. 
       [Curr Opin Rheumatol. 2003]
     * ReviewThe autoinflammatory syndromes.
       Curr Opin Allergy Clin Immunol. 2002 Dec; 2(6):511-6. 
       [Curr Opin Allergy Clin Immunol. 2002]
     * Regulation of cryopyrin/Pypaf1 signaling by pyrin, the familial
       Mediterranean fever gene product.
       Biochem Biophys Res Commun. 2003 Mar 14; 302(3):575-80. 
       [Biochem Biophys Res Commun. 2003]
     * The SPRY domain of Pyrin, mutated in familial Mediterranean fever
       patients, interacts with inflammasome components and inhibits
       proIL-1beta processing.
       Cell Death Differ. 2007 Aug; 14(8):1457-66. Epub 2007 Apr 13.
       [Cell Death Differ. 2007]
     * Mutation of a new gene encoding a putative pyrin-like protein
       causes familial cold autoinflammatory syndrome and Muckle-Wells
       syndrome.
       Nat Genet. 2001 Nov; 29(3):301-5. 
       [Nat Genet. 2001]
     * » See reviews... | » See all...
Cited by 2 PubMed Central articles
     * Osteoblasts express NLRP3, a nucleotide-binding domain and
       leucine-rich repeat region containing receptor implicated in
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       McCall SH, Sahraei M, Young AB, Worley CS, Duncan JA, Ting JP,
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     * Severe disease in patients with rheumatoid arthritis carrying a
       mutation in the Mediterranean fever gene.
       Rabinovich E, Livneh A, Langevitz P, Brezniak N, Shinar E, Pras M,
       Shinar Y. Ann Rheum Dis. 2005 Jul; 64(7):1009-14. 
       [Ann Rheum Dis. 2005]
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