title: Interleukin-2-dependent T-cell lines established from paroxysmal nocturnal hemoglobinuria patients.
abstract: Peripheral blood T lymphocytes obtained from two patients with paroxysmal nocturnal hemoglobinuria (PNH) were immortalized with human T-lymphotropic virus type 1 (HTLV-1). These cells showed interleukin-2 (IL-2)-dependent cell growth in culture. Cell surface analysis showed that they had the phenotype of a helper/inducer T subset that was positive for CD2, CD3, and CD4, but negative for CD8, similar to adult T-cell leukemia cells induced by HTLV-1. These cell lines lacked glycosylphosphatidylinositol (GPI)-anchored proteins, CDw52, CD55 (decay-accelerating factor; DAF), and CD59 on the cell surface, whereas intracellular DAF protein was detected. These T-subset cell lines with a PNH phenotype did not synthesize GPI anchor, whereas a control cell line, similarly prepared from the T cells of a healthy volunteer, produced the anchor. The control cells expressed CDw52, DAF, and CD59 on the cell surface and showed the phenotype of a helper/inducer subset. Southern blot analysis confirmed the clonality of each cell line. These CD4+ T-cell lines with a PNH phenotype and a subset-matched control counterpart could be a useful model for PNH investigation.
authors.affiliation: Second Department of Internal Medicine, Kumamoto University School of Medicine, Japan.
authors.forename: H
authors.initials: H
authors.lastname: Nakakuma
authors.forename: S
authors.initials: S
authors.lastname: Nagakura
authors.forename: K
authors.initials: K
authors.lastname: Horikawa
authors.forename: M
authors.initials: M
authors.lastname: Hidaka
authors.forename: T
authors.initials: T
authors.lastname: Kawaguchi
authors.forename: N
authors.initials: N
authors.lastname: Iwamoto
authors.forename: I
authors.initials: I
authors.lastname: Sanada
authors.forename: T
authors.initials: T
authors.lastname: Kagimoto
authors.forename: K
authors.initials: K
authors.lastname: Takatsuki
mesh_terms: D015703:Antigens, CD / Q000032:analysis; D018958:CD55 Antigens; D002460:Cell Line; D017261:Glycosylphosphatidylinositols / Q000096:biosynthesis; D006457:Hemoglobinuria, Paroxysmal / Q000276:immunology*; D015368:Human T-lymphotropic virus 1 / Q000235:genetics; D006801:Humans; D007376:Interleukin-2 / Q000494:pharmacology*; D007621:Karyotyping; D008213:Lymphocyte Activation / Q000187:drug effects; D008562:Membrane Glycoproteins / Q000032:analysis; D013601:T-Lymphocytes / Q000502:physiology*
chemical_list: D015703:Antigens, CD; D018958:CD55 Antigens; D017261:Glycosylphosphatidylinositols; D007376:Interleukin-2; D008562:Membrane Glycoproteins