INVITRO STUDIES ON PROTOPORPHYRIN UPTAKE AND PHOTOSENSITIVITY OF NORMAL SKIN FIBROBLASTS AND FIBROBLASTS FROM PATIENTS WITH ERYTHROPOIETIC PROTOPORPHYRIA

[摘要] Fibroblasts derived from patients with erythropoietic protoporphyria (EPP) are not sensitive to violet light and do not contain an excess of protoporphyrin (PP). Fibroblasts from both normal individuals and patients with EPP can take up very low concentrations of PP from culture medium. Cells grown in PP-containing medium showed a gradually increasing but limited uptake of PP and also an increased sensitivity to light. A sensitive scanning microfluorometric method has made it possible to demonstrate that the PP is mainly localized in the perinuclear granules. After exposure to violet light, cells photosensitized by PP in a culture medium showed increased membrane permeability as well as reduced reproductive capacity. Both of these photodamage effects can be repaired during postirradiation incubation in culture medium at 37.degree. C.