title: Establishment of a human T-cell line with deficient surface expression of glycosylphosphatidylinositol (GPI)-anchored proteins from a patient with paroxysmal nocturnal haemoglobinuria.
abstract: A novel interleukin-2 dependent T-cell line, PMT-2Y, was established from the peripheral blood of a patient with paroxysmal nocturnal haemoglobinuria (PNH) by human T lymphotropic virus type I (HTLV-I)-mediated transformation. PMT-2Y cells are positive for CD2, CD3, CD4, CD25, T cell receptor alpha beta and HLA-DR, but negative for CD1, CD7, CD8, CD19 and CD20, indicating that the clone belongs to a helper/inducer subset of T cells. PMT-2Y cells have the monoclonal integration of HTLV-I proviral DNA, suggesting that they derived from a single clone. Moreover, they lack surface expression of complement regulatory proteins such as DAF (CD55) and CD59, that are the most important glycosylphosphatidylinositol (GPI)-anchored membrane proteins defective in haemopoietic cells of patients with PNH. Northern blot analysis, however, revealed the production of normal levels of DAF mRNAs. Thus, PMT-2Y is derived from a PNH T cell clone and may be a useful model to study PNH.
authors.affiliation: Second Department of Internal Medicine, Kumamoto University School of Medicine, Japan.
authors.forename: T
authors.initials: T
authors.lastname: Masuda
authors.forename: Y
authors.initials: Y
authors.lastname: Yonemura
authors.forename: K
authors.initials: K
authors.lastname: Fujimoto
authors.forename: M
authors.initials: M
authors.lastname: Hidaka
authors.forename: S
authors.initials: S
authors.lastname: Nagakura
authors.forename: H
authors.initials: H
authors.lastname: Nakakuma
authors.forename: H
authors.initials: H
authors.lastname: Hata
authors.forename: I
authors.initials: I
authors.lastname: Sanada
authors.forename: M
authors.initials: M
authors.lastname: Kawakita
authors.forename: K
authors.initials: K
authors.lastname: Takatsuki
mesh_terms: D015703:Antigens, CD / Q000097:blood* / Q000235:genetics; D000954:Antigens, Surface / Q000032:analysis; D001483:Base Sequence; D018958:CD55 Antigens; D002460:Cell Line / Q000166:cytology / Q000276:immunology*; D002469:Cell Separation; D002472:Cell Transformation, Viral; D002999:Clone Cells / Q000276:immunology; D003169:Complement Inactivator Proteins / Q000032:analysis*; D005260:Female; D017261:Glycosylphosphatidylinositols / Q000097:blood; D006457:Hemoglobinuria, Paroxysmal / Q000276:immunology*; D015368:Human T-lymphotropic virus 1; D006801:Humans; D008562:Membrane Glycoproteins / Q000097:blood* / Q000235:genetics; D008875:Middle Aged; D008969:Molecular Sequence Data; D012333:RNA, Messenger / Q000032:analysis; D006377:T-Lymphocytes, Helper-Inducer / Q000276:immunology*
chemical_list: D015703:Antigens, CD; D000954:Antigens, Surface; D018958:CD55 Antigens; D003169:Complement Inactivator Proteins; D017261:Glycosylphosphatidylinositols; D008562:Membrane Glycoproteins; D012333:RNA, Messenger