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The apoptosis modulating role of SAP (SLAM associated protein) contributes to the symptomatology of the X linked lymphoproliferative disease
Department of Microbiology; Tumor and Cell Biology (MTC); Karolinska Institute; Stockholm, Sweden.
Department of Microbiology; Tumor and Cell Biology (MTC); Karolinska Institute; Stockholm, Sweden.
Uppsala University, Disciplinary Domain of Medicine and Pharmacy, Medicinska och farmaceutiska vetenskapsområdet, centrumbildningar mm , Ludwig Institute for Cancer Research.
Department of Microbiology; Tumor and Cell Biology (MTC); Karolinska Institute; Stockholm, Sweden.
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2009 (English)In: Cell Cycle, ISSN 1538-4101, E-ISSN 1551-4005, Vol. 8, no 19, 3086-3090 p.Article in journal (Refereed) Published
Abstract [en]

Deletion or mutation of the SH2D1A gene located at Xq25 is responsible for the development of the X-linked lymphoproliferative disease, XLP. Primary infection of the affected individuals with EBV leads to fulminant and often fatal infectious mononucleosis, FIM. Moreover, they run a 200 fold elevated risk for lymphoma development. Due to the critical role of the immune response for the outcome of EBV infection and the detection of EBV genomes in several malignancies, XLP studies have been mainly focused on the immunological aspects. The involvement of SAP in the apoptotic machinery provides a further aspect in the complex syndrome of XLP. Functional impairment of SAP leads to defective apoptotic responses. Activation induced apoptosis plays a pivotal role in the termination of the lymphocyte proliferation in IM. This mechanism is inefficient in XLP patients. In addition, in the absence of SAP, lymphoma development may be promoted by the illegitimate survival of lymphocytes with damaged DNA that would be normally eliminated by apoptosis.

Place, publisher, year, edition, pages
Landes Bioscience , 2009. Vol. 8, no 19, 3086-3090 p.
Keyword [en]
XLP, SAP, EBV, apoptosis, B cell lymphoma, infectious mononucleosis
National Category
Medical and Health Sciences
Identifiers
URN: urn:nbn:se:uu:diva-113662ISI: 000270495500013PubMedID: 19738428OAI: oai:DiVA.org:uu-113662DiVA: diva2:291587
Available from: 2010-02-02 Created: 2010-02-02 Last updated: 2017-12-12Bibliographically approved

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