title: Herpes virus saimiri transformation of T cells in CD3 gamma immunodeficiency: phenotypic and functional characterization.
abstract: The characterization of T cell immunodeficiencies could in part be supported by using stable cell lines in which biochemical and molecular studies of the defect could be carried out thereby omitting frequent bleeding of patients. First attempts to obtain such cell lines included HTLV-I transformation and exogenous IL-2 administration, but both models have important disadvantages. Recently, a virus isolated from the squirrel monkey, Herpes virus saimiri (HVS), has been reported to have the ability to transform T cells. A stable IL-2-dependent HVS-transformed T cell line from a CD3 gamma deficient patient has been obtained; and this cell line displays both the phenotypic and the functional characteristics of the patient's lymphocytes. Moreover, the line down-modulates TCR/CD3 surface expression upon CD3 engagement, as do the patient's lymphocytes, showing that CD3 gamma and its phosphorylation are not necessary for TCR/CD3 internalization. In addition, the abnormal staining pattern of different anti-TCR/CD3 monoclonal antibodies is preserved in the HVS-patient line. Since HVS is capable of transforming CD3 gamma- T cells, the CD3 gamma chain does not seem to be involved in the HVS receptor process. The fact that it is not possible to obtain a CD8+ HVS line from the CD3 gamma- patient supports the existence of a functional anomaly in his scanty CD8+ peripheral lymphocytes. Thus, HVS transformation is a suitable model for T cell immunodeficiency studies and characterization. It may also be used in the future in cellular models for in vitro gene therapy trials.
authors.affiliation: Department of Immunology, Hospital Universitario 12 de Octubre, Madrid, Spain.
authors.forename: C
authors.initials: C
authors.lastname: Rodríguez-Gallego
authors.forename: A
authors.initials: A
authors.lastname: Corell
authors.forename: A
authors.initials: A
authors.lastname: Pacheco
authors.forename: M
authors.initials: M
authors.lastname: Timón
authors.forename: J R
authors.initials: JR
authors.lastname: Regueiro
authors.forename: L M
authors.initials: LM
authors.lastname: Allende
authors.forename: A
authors.initials: A
authors.lastname: Madroño
authors.forename: A
authors.initials: A
authors.lastname: Arnaiz-Villena
mesh_terms: D017252:CD3 Complex / Q000032:analysis* / Q000235:genetics; D002461:Cell Line, Transformed; D002472:Cell Transformation, Viral / Q000276:immunology*; D005434:Flow Cytometry; D006567:Herpesvirus 2, Saimiriine / Q000276:immunology*; D006801:Humans; D007153:Immunologic Deficiency Syndromes / Q000276:immunology*; D016130:Immunophenotyping; D008213:Lymphocyte Activation; D013601:T-Lymphocytes / Q000276:immunology* / Q000821:virology*
chemical_list: D017252:CD3 Complex