Use of lymphoblastoid cell lines to evaluate the hypersensitivity to ultraviolet radiation in Cockayne syndrome

Journal Article · Tue May 01 04:00:00 EDT 1984 · J. Invest. Dermatol.; (United States)

DOI:https://doi.org/10.1111/1523-1747.ep12260999· OSTI ID:5707925

Otsuka, F; Tarone, R E; Cayeux, S; Robbins, J H

Cockayne syndrome (CS) is a rare autosomal recessive disease characterized by acute sun sensitivity, cachectic dwarfism, and neurologic and skeletal abnormalities. Cultured skin fibroblasts from patients with this disease are known to be hypersensitive to the lethal effects of 254-nm UV radiation. The authors have studied the sensitivity of 254-nm UV radiation of lymphoblastoid lines derived from 3 typical CS patients, 1 atypical CS patient who had a very late age of onset of clinical manifestations, 2 patients who had both xeroderma pigmentosum (XP) and typical CS, and 3 heterozygous parents of these patients. Post-UV survival was determined by the trypan-blue dye-exclusion method. The lymphoblastoid lines from the 3 typical CS patients, the atypical CS patient, and the 2 patients with both CS and XP had decreased post-UV viability in comparison with lines from normal donors. Lines from the heterozygous parents had normal post-UV viability. The post-UV viability of the typical CS lines was similar to that of a XP complementation group C line. The relative post-UV viability of lymphoblastoid lines from the typical CS patients was similar to the relative post-UV survival of their fibroblast lines. The lymphoblastoid line from the atypical CS patient had a post-UV viability similar to that of the typical CS patients. Thus, the relative hypersensitivity of CS patients cells in vitro does not reflect the severity or age of onset of the patients clinical manifestations. The lymphoblastoid lines from the 2 patients who had both CS and XP were significantly more sensitive to the UV radiation than those from patients with only CS. Our studies demonstrate that lymphoblastoid lines from patients with CS are appropriate and useful cell lines for the study of the inherited hypersensitivity to UV radiation.

• Research Organization: National Cancer Inst., Bethesda, MD
• OSTI ID: 5707925
• Journal Information: J. Invest. Dermatol.; (United States), Journal Name: J. Invest. Dermatol.; (United States) Vol. 82:5; ISSN JIDEA
• Country of Publication: United States
• Language: English

Related Subjects

560121* -- Radiation Effects on Cells-- External Source-- (-1987)
63 RADIATION, THERMAL, AND OTHER ENVIRON. POLLUTANT EFFECTS ON LIVING ORGS. AND BIOL. MAT.
AGE GROUPS
ANIMAL CELLS
BIOLOGICAL EFFECTS
BIOLOGICAL MATERIALS
BIOLOGICAL RADIATION EFFECTS
BLOOD
BLOOD CELLS
BODY FLUIDS
CELL CULTURES
CHILDREN
COMPARATIVE EVALUATIONS
CONGENITAL DISEASES
CONNECTIVE TISSUE CELLS
DISEASES
DOSE-RESPONSE RELATIONSHIPS
ELECTROMAGNETIC RADIATION
LEUKOCYTES
LYMPHOCYTES
MATERIALS
PATIENTS
PHENOTYPE
RADIATION EFFECTS
RADIATIONS
RADIOSENSITIVITY
RADIOSENSITIVITY EFFECTS
SOMATIC CELLS
TOLERANCE
ULTRAVIOLET RADIATION