2001
DOI: 10.1146/annurev.immunol.19.1.657
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X-Linked Lymphoproliferative Disease: A Progressive Immunodeficiency

Abstract: Our understanding of the X-linked lymphoproliferative syndrome (XLP) has advanced significantly in the last two years. The gene that is altered in the condition (SAP/SH2D1A) has been cloned and its protein crystal structure solved. At least two sets of target molecules for this small SH2 domain-containing protein have been identified: A family of hematopoietic cell surface receptors, i.e. the SLAM family, and a second molecule, which is a phosphorylated adapter. A SAP-like protein, EAT-2, has also been found t… Show more

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Cited by 202 publications
(163 citation statements)
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“…Lymphocytes recovered in patients undergoing allogeneic BMT were analyzed at different time intervals, starting from engraftment (occurring at day [12][13][14][15][16][17][18][19][20].…”
Section: Surface Nk Receptors Expressed By Nk Cells After Bmtmentioning
confidence: 99%
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“…Lymphocytes recovered in patients undergoing allogeneic BMT were analyzed at different time intervals, starting from engraftment (occurring at day [12][13][14][15][16][17][18][19][20].…”
Section: Surface Nk Receptors Expressed By Nk Cells After Bmtmentioning
confidence: 99%
“…As a consequence SHP-1 phosphatase can be recruited by 2B4 itself. Remarkably, the inhibitory 2B4 may provide an efficient fail-safe mechanism in cells that don't yet express inhibitory receptors specific for HLA class I molecules [11,12].…”
Section: Introductionmentioning
confidence: 99%
“…Hastalıklı erkekler EBV'ye bu kadar duyarlı olmasına karşın, diğer herpes ve herpes dışı virüslere ve bakteriyel patojenlere normal immün yanıt verirler (9)(10)(11).…”
Section: ö öZ Ze Et Tunclassified
“…CD8+ ve CD4+ T lenfositlerdeki ekspresyonu aktivasyonla artar (2,9,21). 2B4, MHC Klas 1'den bağımsız olarak, NK hücre aktivasyonunu düzenler.…”
Section: ö öZ Ze Et Tunclassified
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