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    Inflammation in mice ectopically expressing human Pyogenic Arthritis, Pyoderma Gangrenosum, and Acne (PAPA) Syndrome-associated PSTPIP1 A230T mutant proteins

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    Authors
    Wang, Donghai
    Hoing, Susanne
    Patterson, Heide Christine
    Ahmad, Umtul M.
    Rathinam, Vijay A. K.
    Rajewsky, Klaus
    Fitzgerald, Katherine A.
    Golenbock, Douglas T.
    UMass Chan Affiliations
    Department of Medicine, Division of Infectious Diseases and Immunology
    Document Type
    Journal Article
    Publication Date
    2013-02-15
    Keywords
    Acne Vulgaris
    Adaptor Proteins, Signal Transducing
    Alleles
    Animals
    Arthritis, Infectious
    Autoimmune Diseases
    Caspase 1
    Cytokines
    Cytoskeletal Proteins
    Gene Expression Regulation
    Humans
    Immunity, Innate
    Inflammation
    Interleukin-1beta
    Mice
    Mice, Inbred C57BL
    Mice, Knockout
    *Mutation
    Phenotype
    Pyoderma Gangrenosum
    Signal Transduction
    Syndrome
    Turpentine
    Biochemistry
    Immunology and Infectious Disease
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    Link to Full Text
    http://dx.doi.org/10.1074/jbc.M112.443077
    Abstract
    Pyogenic Arthritis, Pyoderma Gangrenosum, and Acne Syndrome (PAPA syndrome) is an autoinflammatory disease caused by aberrant production of the proinflammatory cytokine interleukin-1. Mutations in the gene encoding proline serine threonine phosphatase-interacting protein-1 (PSTPIP1) have been linked to PAPA syndrome. PSTPIP1 is an adaptor protein that interacts with PYRIN, the protein encoded by the Mediterranean Fever (MEFV) gene whose mutations cause Familial Mediterranean Fever (FMF). However, the pathophysiological function of PSTPIP1 remains to be elucidated. We have generated mouse strains that either are PSTPIP1 deficient or ectopically express mutant PSTPIP1. Results from analyzing these mice suggested that PSTPIP1 is not an essential regulator of the Nlrp3, Aim2, or Nlrc4 inflammasomes. Although common features of human PAPA syndrome such as pyogenic arthritis and skin inflammation were not recapitulated in the mouse model, ectopic expression of the mutant but not the wild type PSTPIP1 in mice lead to partial embryonic lethality, growth retardation, and elevated level of circulating proinflammatory cytokines.
    Source
    J Biol Chem. 2013 Feb 15;288(7):4594-601. doi: 10.1074/jbc.M112.443077. Link to article on publisher's site
    DOI
    10.1074/jbc.M112.443077
    Permanent Link to this Item
    http://hdl.handle.net/20.500.14038/29622
    PubMed ID
    23293022
    Related Resources
    Link to Article in PubMed
    ae974a485f413a2113503eed53cd6c53
    10.1074/jbc.M112.443077
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