title: Elevated chromosome aberration frequency after X-ray exposure of cultured fibroblasts derived from patients with porokeratosis.
abstract: Porokeratosis (PK) is a rare genetic skin disorder inherited as an autosomal dominant trait and regarded as a disease predisposing to cancer. To evaluate chromosomal radiosensitivity of PK cells, we examined chromosome aberration frequency after X-irradiation of cultured skin fibroblasts derived from PK patients and controls. Without X-ray exposure, frequencies of chromosome-type aberrations (exchanges or deletions) were not different between the patients and controls. Following X-ray irradiation, frequencies of deletions in the patient group were significantly increased, whereas those of exchanges were not elevated. No differences in chromatid-type aberration frequency were found between the patients and controls with or without exposure to X-ray. The observed radiosensitivity, though not as high as in ataxia telangiectasia (AT) cells, agrees well with the previously reported higher radiosensitivity of PK fibroblasts in survival analysis.
authors.affiliation: Department of Health Sciences, Yamanashi Medical University, Japan.
authors.forename: T
authors.initials: T
authors.lastname: Takeshita
authors.forename: M
authors.initials: M
authors.lastname: Higurashi
authors.forename: C
authors.initials: C
authors.lastname: Ariizumi-Shibusawa
authors.forename: K
authors.initials: K
authors.lastname: Shimizu
authors.forename: S
authors.initials: S
authors.lastname: Iijima
authors.forename: Z
authors.initials: Z
authors.lastname: Yamagata
authors.forename: A
authors.initials: A
authors.lastname: Asaka
authors.forename: K
authors.initials: K
authors.lastname: Morimoto
authors.forename: Y
authors.initials: Y
authors.lastname: Ishibashi
authors.forename: F
authors.initials: F
authors.lastname: Otsuka
mesh_terms: D000704:Analysis of Variance; D002872:Chromosome Deletion; D005260:Female; D005347:Fibroblasts / Q000528:radiation effects*; D006801:Humans; D008297:Male; D008875:Middle Aged; D017499:Porokeratosis / Q000235:genetics* / Q000473:pathology; D014178:Translocation, Genetic