TcR-α/β+ CD4-CD8- T cells in humans with the autoimmune lymphoproliferative syndrome express a novel CD45 isoform that is analogous to murine B220 and represents a marker of altered O-glycan biosynthesis

Academic Article


  • Autoimmune lymphoproliferative syndrome (ALPS), caused by inherited defects in apoptosis secondary to mutations in genes encoding Fas/CD95/APO-1 and Fas ligand (Fasl)/CD95L, is characterized by nonmalignant lymphadenopathy and splenomegaly, increased T cell receptor α/β+ CD4-CD8- T cells (α/β+ double-negative T cells [α/β+-DNT cells]), autoimmunity, hypergammaglobulinemia, and cytokine abnormalities. The α/β+-DNT cells are immunophenotypically and functionally similar to α/β+-DNT cells that accumulate in lpr and gld mice, which bear genetic mutations in Fas and FasL. In these mice, α/β+-DNT cells express the B-cell-specific CD45R isoform B220. We show that α/β+-DNT cells of ALPS patients, with either Fas or FasL mutations, also express B220. In addition, also similar to LPR/gLD mice, they have an unusual population of B220-positive CD4+ T cells. B220 expression, together with our finding of characteristic lectin binding profiles, demonstrates that cell surface O-linked glycoproteins have undergone specific modifications, which may have consequences for lymphocyte trafficking, cell-cell interactions, and access to alternative apoptosis pathways. © 2001 Academic Press.
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    Digital Object Identifier (doi)

    Author List

  • Bleesing JJH; Brown MR; Dale JK; Straus SE; Lenardo MJ; Puck JM; Atkinson TP; Fleisher TA
  • Start Page

  • 314
  • End Page

  • 324
  • Volume

  • 100
  • Issue

  • 3