Establishment of a human T-cell line with deficient surface expression of glycosylphosphatidylinositol (GPI)-anchored proteins from a patient with paroxysmal nocturnal haemoglobinuria.
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.
Masuda
T
T
Second Department of Internal Medicine, Kumamoto University School of Medicine, Japan.
Yonemura
Y
Y
Fujimoto
K
K
Hidaka
M
M
Nagakura
S
S
Nakakuma
H
H
Hata
H
H
Sanada
I
I
Kawakita
M
M
Takatsuki
K
K
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*
D015703:Antigens, CD; D000954:Antigens, Surface; D018958:CD55 Antigens; D003169:Complement Inactivator Proteins; D017261:Glycosylphosphatidylinositols; D008562:Membrane Glycoproteins; D012333:RNA, Messenger