Statements in which the resource exists as a subject.
PredicateObject
rdf:type
lifeskim:mentions
pubmed:issue
6
pubmed:dateCreated
2009-6-22
pubmed:abstractText
NLRP3 nucleates the inflammasome, a protein complex responsible for cleavage of prointerleukin-1beta (IL-1beta) to its active form. Mutations in the NLRP3 gene cause the autoinflammatory disease spectrum cryopyrin-associated periodic syndromes (CAPS). The central role of IL-1beta in CAPS is supported by the response to IL-1-targeted therapy. We developed two Nlrp3 mutant knockin mouse strains to model CAPS to examine the role of other inflammatory mediators and adaptive immune responses in an innate immune-driven disease. These mice had systemic inflammation and poor growth, similar to some human CAPS patients, and demonstrated early mortality, primarily mediated by myeloid cells. Mating these mutant mice to various gene mutant backgrounds showed that the mouse disease phenotype required an intact inflammasome, was only partially dependent on IL-1beta, and was independent of T cells. These data suggest that CAPS are true inflammasome-mediated diseases and provide insight for more common inflammatory disorders.
pubmed:grant
pubmed:commentsCorrections
http://linkedlifedata.com/resource/pubmed/commentcorrection/19501000-10621974, http://linkedlifedata.com/resource/pubmed/commentcorrection/19501000-11971877, http://linkedlifedata.com/resource/pubmed/commentcorrection/19501000-12667444, http://linkedlifedata.com/resource/pubmed/commentcorrection/19501000-14634131, http://linkedlifedata.com/resource/pubmed/commentcorrection/19501000-15030775, http://linkedlifedata.com/resource/pubmed/commentcorrection/19501000-1547488, http://linkedlifedata.com/resource/pubmed/commentcorrection/19501000-15541451, http://linkedlifedata.com/resource/pubmed/commentcorrection/19501000-15967716, http://linkedlifedata.com/resource/pubmed/commentcorrection/19501000-16407888, http://linkedlifedata.com/resource/pubmed/commentcorrection/19501000-16407889, http://linkedlifedata.com/resource/pubmed/commentcorrection/19501000-17164409, http://linkedlifedata.com/resource/pubmed/commentcorrection/19501000-17964261, http://linkedlifedata.com/resource/pubmed/commentcorrection/19501000-18400188, http://linkedlifedata.com/resource/pubmed/commentcorrection/19501000-18668535, http://linkedlifedata.com/resource/pubmed/commentcorrection/19501000-18827826, http://linkedlifedata.com/resource/pubmed/commentcorrection/19501000-19362022, http://linkedlifedata.com/resource/pubmed/commentcorrection/19501000-19538926, http://linkedlifedata.com/resource/pubmed/commentcorrection/19501000-3332485, http://linkedlifedata.com/resource/pubmed/commentcorrection/19501000-9317135, http://linkedlifedata.com/resource/pubmed/commentcorrection/19501000-9504047, http://linkedlifedata.com/resource/pubmed/commentcorrection/19501000-9553774
pubmed:language
eng
pubmed:journal
pubmed:citationSubset
IM
pubmed:chemical
pubmed:status
MEDLINE
pubmed:month
Jun
pubmed:issn
1097-4180
pubmed:author
pubmed:issnType
Electronic
pubmed:day
19
pubmed:volume
30
pubmed:owner
NLM
pubmed:authorsComplete
Y
pubmed:pagination
875-87
pubmed:dateRevised
2011-6-1
pubmed:meshHeading
pubmed:year
2009
pubmed:articleTitle
Inflammasome-mediated disease animal models reveal roles for innate but not adaptive immunity.
pubmed:affiliation
Genetics and Genomics Branch, National Institute of Arthritis and Musculoskeletal and Skin Diseases, National Institutes of Health, Bethesda, MD 20892, USA.
pubmed:publicationType
Journal Article, Research Support, Non-U.S. Gov't, Research Support, N.I.H., Extramural