The SPRY domain of Pyrin, mutated in familial Mediterranean fever patients, interacts with inflammasome components and inhibits proIL-1beta processing.

Details

Serval ID
serval:BIB_24E61CE732DE
Type
Article: article from journal or magazin.
Collection
Publications
Institution
Title
The SPRY domain of Pyrin, mutated in familial Mediterranean fever patients, interacts with inflammasome components and inhibits proIL-1beta processing.
Journal
Cell Death and Differentiation
Author(s)
Papin S., Cuenin S., Agostini L., Martinon F., Werner S., Beer H.D., Grütter C., Grütter M., Tschopp J.
ISSN
1350-9047 (Print)
ISSN-L
1350-9047
Publication state
Published
Issued date
2007
Volume
14
Number
8
Pages
1457-1466
Language
english
Notes
Publication types: In Vitro ; Journal Article ; Research Support, Non-U.S. Gov't
Publication Status: ppublish
Abstract
The autoinflammatory disorders Muckle-Wells syndrome, familial cold urtecaria and chronic infantile neurological cutaneous and articular syndrome are associated with mutations in the NALP3 (Cryopyrin) gene, which is the central platform of the proinflammatory caspase-1 activating complex, named the inflammasome. In patients with another autoinflammatory disorder, familial Mediterranean fever (FMF), mutations in the SPRY domain of the Pyrin protein are frequently found. Recent evidence suggests that Pyrin associates with ASC, an inflammasome component, via its Pyrin domain, thereby halting the inflammatory response. This interaction, however, does not explain the effects of mutations of the SPRY domain found in FMF patients. Here we show that the Pyrin SPRY domain not only interacts with NALP3, but also with caspase-1 and its substrate pro-interleukin(IL)-1beta. Whereas a Pyrin knockdown results in increased caspase-1 activation and IL-1beta secretion, overexpression of the SPRY domain alone blocks these processes. Thus Pyrin binds to several inflammasome components thereby modulating their activity.
Keywords
Autoimmunity, Base Sequence, Carrier Proteins/metabolism, Caspase 1/metabolism, Caspase Inhibitors, Cell Line, Cytoskeletal Proteins/chemistry, Cytoskeletal Proteins/genetics, DNA/genetics, Familial Mediterranean Fever/genetics, Familial Mediterranean Fever/immunology, Humans, Interleukin-1/metabolism, Models, Biological, Mutation, Protein Binding, Protein Precursors/metabolism, Protein Processing, Post-Translational, Protein Structure, Tertiary, Recombinant Proteins/chemistry, Recombinant Proteins/genetics, Transfection
Pubmed
Web of science
Open Access
Yes
Create date
24/01/2008 16:19
Last modification date
20/08/2019 14:03
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