The gene defective in X-linked lymphoproliferative disease controls T cell dependent immune surveillance against Epstein-Barr virus.

Abstract:

:Our understanding of the X-linked lymphoproliferative syndrome (XLP) has advanced significantly in the past two years. The gene that is aberrant in the condition - SH2D1A/SAP, which encodes SAP (signaling lymphocytic activation molecule [SLAM]-associated protein) - was cloned, the crystal structure of its product was solved and insights into the signaling mechanisms of this small SH2-domain-containing protein via the cell surface receptors SLAM and 2B4 have been provided. SAP mutation, and not Epstein-Barr virus infection per se, may be critical for XLP.

journal_name

Curr Opin Immunol

authors

Howie D,Sayos J,Terhorst C,Morra M

doi

10.1016/s0952-7915(00)00123-0

subject

Has Abstract

pub_date

2000-08-01 00:00:00

pages

474-8

issue

4

eissn

0952-7915

issn

1879-0372

pii

S0952-7915(00)00123-0

journal_volume

12

pub_type

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