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Common expression of an unusual CD45 isoform on T cells from patients with large granular lymphocyte leukaemiaand autoimmune lymphoproliferative syndrome
Author(s) -
Bleesing Jack J. H.,
Janik John E.,
Fleisher Thomas A.
Publication year - 2003
Publication title -
british journal of haematology
Language(s) - English
Resource type - Journals
SCImago Journal Rank - 1.907
H-Index - 186
eISSN - 1365-2141
pISSN - 0007-1048
DOI - 10.1046/j.1365-2141.2003.04034.x
Subject(s) - cytotoxic t cell , autoimmunity , gene isoform , immunology , autoimmune lymphoproliferative syndrome , t lymphocyte , protein tyrosine phosphatase , biology , t cell , lymphocyte , immune system , apoptosis , microbiology and biotechnology , programmed cell death , fas receptor , genetics , in vitro , signal transduction , gene
Summary. Patients with T‐cell large granular lymphocyte (T‐LGL) leukaemia and autoimmune lymphoproliferative syndrome (ALPS) share many features, including autoimmunity and an expansion of (cytotoxic) T cells, which in ALPS patients express an unusual (B220) isoform of CD45, corresponding to an altered O ‐glycosylation profile. Here we showed that T‐LGL leukaemia cells also expressed this B220 isoform. We hypothesize that B220 + T cells constitute proliferating T cells that have become competent to undergo apoptosis, but that constitutive (ALPS) or functional (T‐LGL) defects prevent this process. Altered O ‐glycosylation of the extracellular domains of CD45 may have consequences for this tyrosine phosphatase as a regulator of cell proliferation and survival.

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