Document Detail

How do SAP family deficiencies compromise immunity?
MedLine Citation:
PMID:  20650688     Owner:  NLM     Status:  MEDLINE    
The signaling lymphocytic activation molecule (SLAM)-associated protein (SAP) family of adaptors couples SLAM family receptors to activating intracellular signaling pathways that drive immune cell activation or differentiation. In the absence of SAP family adaptors, SLAM family receptors become inhibitory, possibly through coupling to the Src-homology-2-containing phosphatases. This "switch-of-function" of SLAM family receptors provides an explanation for the severe immune dysfunctions observed in humans with X-linked lymphoproliferative disease due to SAP deficiency, as well as in genetically engineered mice that lack SAP family adaptors.
Zhongjun Dong; André Veillette
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Publication Detail:
Type:  Journal Article; Research Support, Non-U.S. Gov't; Review     Date:  2010-07-23
Journal Detail:
Title:  Trends in immunology     Volume:  31     ISSN:  1471-4981     ISO Abbreviation:  Trends Immunol.     Publication Date:  2010 Aug 
Date Detail:
Created Date:  2010-08-06     Completed Date:  2010-09-20     Revised Date:  -    
Medline Journal Info:
Nlm Unique ID:  100966032     Medline TA:  Trends Immunol     Country:  England    
Other Details:
Languages:  eng     Pagination:  295-302     Citation Subset:  IM    
Copyright Information:
2010 Elsevier Ltd. All rights reserved.
Clinical Research Institute of Montréal, Québec, Canada.
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MeSH Terms
Cell Differentiation
Intracellular Signaling Peptides and Proteins / deficiency,  immunology*
Lymphocytes / cytology,  immunology
Signal Transduction
src Homology Domains
Grant Support
//Canadian Institutes of Health Research; //Howard Hughes Medical Institute
Reg. No./Substance:
0/Intracellular Signaling Peptides and Proteins

From MEDLINE®/PubMed®, a database of the U.S. National Library of Medicine

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