title: Increase in nucleoli after x-radiation of fibroblasts of patients with Gorlin syndrome.
abstract: Gorlin syndrome (GS) is an autosomal dominant disorder in which patients are abnormally susceptible to ionizing radiation with radiotherapeutic doses. Radiogenic basal cell carcinomas may develop with a short latent period in patients. The mechanisms underlying the abnormal radiosusceptibility of cells in patients with GS has not been well characterized. In this study we report an increase in the number of nucleoli in fibroblast cells from 3 patients with GS after x-radiation. In GS fibroblasts, the increase in nucleolus number concomitant with the increase of ribonucleoprotein immunoreactive aggregates within the nucleus was observed after x-radiation, whereas significant change was not found in normal fibroblasts derived from healthy donors. This increase disappeared when cells were cultured with the RNA synthesis inhibitor actinomycin D after x-radiation but not when they were cultured with cycloheximide or aphydicolin, which are protein and DNA synthesis inhibitors, respectively. Ultraviolet exposure did not induce remarkable changes in the GS nucleoli. Thus the increase in nucleoli was induced after x-radiation of GS fibroblasts, and this increase seemed to be related to RNA synthesis metabolism.
authors.affiliation: Department of Ophthalmology, Chiba University School of Medicine, Japan.
authors.forename: M
authors.initials: M
authors.lastname: Dezawa
authors.forename: K
authors.initials: K
authors.lastname: Fujii
authors.forename: K
authors.initials: K
authors.lastname: Kita
authors.forename: J
authors.initials: J
authors.lastname: Nomura
authors.forename: K
authors.initials: K
authors.lastname: Sugita
authors.forename: E
authors.initials: E
authors.lastname: Adachi-Usami
authors.forename: N
authors.initials: N
authors.lastname: Suzuki
mesh_terms: D001478:Basal Cell Nevus Syndrome / Q000378:metabolism* / Q000473:pathology; D002452:Cell Count; D002460:Cell Line; D002466:Cell Nucleolus / Q000378:metabolism* / Q000473:pathology; D003609:Dactinomycin / Q000494:pharmacology; D005347:Fibroblasts / Q000187:drug effects / Q000378:metabolism / Q000528:radiation effects*; D019084:Fluorescent Antibody Technique, Indirect; D006801:Humans; D011003:Ploidies; D011500:Protein Synthesis Inhibitors / Q000494:pharmacology; D012867:Skin / Q000166:cytology / Q000187:drug effects / Q000378:metabolism / Q000528:radiation effects*; D012878:Skin Neoplasms / Q000378:metabolism* / Q000473:pathology; D014466:Ultraviolet Rays; D014965:X-Rays
chemical_list: D011500:Protein Synthesis Inhibitors; D003609:Dactinomycin