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PredicateObject
rdf:type
lifeskim:mentions
pubmed:dateCreated
2011-3-23
pubmed:abstractText
The signaling lymphocyte activation molecule (SLAM)-associated protein, SAP, was first identified as the protein affected in most cases of X-linked lymphoproliferative (XLP) syndrome, a rare genetic disorder characterized by abnormal responses to Epstein-Barr virus infection, lymphoproliferative syndromes, and dysgammaglobulinemia. SAP consists almost entirely of a single SH2 protein domain that interacts with the cytoplasmic tail of SLAM and related receptors, including 2B4, Ly108, CD84, Ly9, and potentially CRACC. SLAM family members are now recognized as important immunomodulatory receptors with roles in cytotoxicity, humoral immunity, autoimmunity, cell survival, lymphocyte development, and cell adhesion. In this review, we cover recent findings on the roles of SLAM family receptors and the SAP family of adaptors, with a focus on their regulation of the pathways involved in the pathogenesis of XLP and other immune disorders.
pubmed:language
eng
pubmed:journal
pubmed:citationSubset
IM
pubmed:chemical
pubmed:status
MEDLINE
pubmed:month
Apr
pubmed:issn
1545-3278
pubmed:author
pubmed:issnType
Electronic
pubmed:day
23
pubmed:volume
29
pubmed:owner
NLM
pubmed:authorsComplete
Y
pubmed:pagination
665-705
pubmed:meshHeading
pubmed:year
2011
pubmed:articleTitle
SLAM family receptors and SAP adaptors in immunity.
pubmed:affiliation
National Human Genome Research Institute, National Institutes of Health, Bethesda, Maryland 20892, USA. jcannons@mail.nih.gov
pubmed:publicationType
Journal Article, Review