Statements in which the resource exists as a subject.
PredicateObject
rdf:type
lifeskim:mentions
pubmed:issue
7
pubmed:dateCreated
2011-5-2
pubmed:abstractText
PAPA syndrome (Pyogenic Arthritis, Pyoderma gangrenosum, and Acne) is an autosomal dominant, hereditary auto-inflammatory disease arising from mutations in the PSTPIP1/CD2BP1 gene on chromosome 15q. These mutations produce a hyper-phosphorylated PSTPIP1 protein and alter its participation in activation of the "inflammasome" involved in interleukin-1 (IL-1?) production. Overproduction of IL-1? is a clear molecular feature of PAPA syndrome. Ongoing research is implicating other biochemical pathways that may be relevant to the distinct pyogenic inflammation of the skin and joints characteristic of this disease. This review summarizes the recent and rapidly accumulating knowledge on these molecular aspects of PAPA syndrome and related disorders.
pubmed:commentsCorrections
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pubmed:language
eng
pubmed:journal
pubmed:status
PubMed-not-MEDLINE
pubmed:month
Nov
pubmed:issn
1875-5488
pubmed:author
pubmed:issnType
Electronic
pubmed:volume
11
pubmed:owner
NLM
pubmed:authorsComplete
Y
pubmed:pagination
519-27
pubmed:dateRevised
2011-7-28
pubmed:year
2010
pubmed:articleTitle
Clinical, Molecular, and Genetic Characteristics of PAPA Syndrome: A Review.
pubmed:affiliation
Sarah M. and Charles E. Seay Center for Musculoskeletal Research, Scottish Rite Hospital for Children, Dallas, Texas 75219.
pubmed:publicationType
Journal Article