IMMUNOCYTOCHEMICAL STUDIES ON THE VIMENTIN DISTRIBUTION AND CELL-PROLIFERATION OF FIBROBLASTS IN PATIENTS WITH FRIEDREICHS ATAXIA

Fibroblasts obtained from patients with Friedreich's ataxia and normal control subjects were studied by immunocytochemistry for intermediate filament vimentin and also for in vitro proliferation. Trypsinized ce lls were seeded on coverslips and incubated for 1.5 h and 24 h. The ex pression of vimentin in cells was investigated by immunofluorescence m icroscopy. Cell proliferation was studied with BrdU antibody technique . Cells from patients with Friedreich's ataxia showed a slower outgrow th of vimentin filaments in comparison to cells from normal controls. These cells also incorporated less 5-bromo-2'-deoxyuridine (BrdU) into their DNA. The observations may be relevant to the clinical manifesta tions of the disease which involves many organs in addition to brain a nd spinal cord.