Lymphoblastoid lines and skin fibroblasts from patients with tuberous sclerosis are abnormally sensitive to ionizing radiation and to a radiomimetic chemical

Journal Article · Mon Mar 01 00:00:00 EST 1982 · J. Invest. Dermatol.; (United States)

DOI:https://doi.org/10.1111/1523-1747.ep12506550· OSTI ID:5475631

Scudiero, D A; Moshell, A N; Scarpinato, R G; Meyer, S A; Clatterbuck, B E; Tarone, R E; Robbins, J H

Lymphoblastoid lines, derived by transforming peripheral blood lymphocytes with Epstein-Barr virus, and skin fibroblast lines were established from two patients with tuberous sclerosis. The number of viable lymphoblastoid cells was determined by their ability to exclude the vital dye trypan blue after their irradiation with x-rays or 254 nm ultraviolet light. The growth of fibroblasts was determined by their ability to form colonies after treatment with the radiomimetic, DNA-damaging chemical N-methyl-N'-nitro-N-nitrosoguanidine. The tuberous sclerosis lymphoblastoid lines were hypersensitive to x-rays but had normal sensitivity to the ultraviolet radiation. The tuberous sclerosis fibroblast lines were hypersensitive to the N-methyl-N'-nitro-N-nitrosoguanidine. The hypersensitivity of tuberous sclerosis cells to x-rays and to N-methyl-N'-nitro-N-nitrosoguanidine is believed to reflect defective repair of DNA damaged by these agents and may provide the basis for in vitro, including prenatal, diagnostic tests for tuberous sclerosis.

• Research Organization: Chemical Carcinogenesis Program, Frederick Cancer Research Center, Maryland
• OSTI ID: 5475631
• Journal Information: J. Invest. Dermatol.; (United States), Vol. 78:3
• Country of Publication: United States
• Language: English

Related Subjects

63 RADIATION, THERMAL, AND OTHER ENVIRON. POLLUTANT EFFECTS ON LIVING ORGS. AND BIOL. MAT.
62 RADIOLOGY AND NUCLEAR MEDICINE
CELL KILLING
RADIOINDUCTION
DNA
BIOLOGICAL REPAIR
FIBROBLASTS
RADIOSENSITIVITY
LYMPHOCYTES
NITROSO COMPOUNDS
BIOLOGICAL EFFECTS
SKELETAL DISEASES
DIAGNOSIS
COLONY FORMATION
EXTREME ULTRAVIOLET RADIATION
PATIENTS
SKIN
X RADIATION
ANIMAL CELLS
BIOLOGICAL MATERIALS
BIOLOGICAL RECOVERY
BLOOD
BLOOD CELLS
BODY
BODY FLUIDS
CONNECTIVE TISSUE CELLS
DISEASES
ELECTROMAGNETIC RADIATION
IONIZING RADIATIONS
LEUKOCYTES
MATERIALS
NUCLEIC ACIDS
ORGANIC COMPOUNDS
ORGANIC NITROGEN COMPOUNDS
ORGANS
RADIATIONS
RECOVERY
REPAIR
SOMATIC CELLS
ULTRAVIOLET RADIATION
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