Ataxia telangiectasia. Evaluation of radiosensitivity in cultured skin fibroblasts as a diagnostic test.

Archives of disease in childhood ·Vol. 53 ·No. 5 ·1978-05-00 ·Pages 386-90

Cox R, Hosking GP, Wilson J

Abstract

The sensitivity to x-ray inactivation of cultured skin fibroblasts from clinically confirmed and suspected cases of ataxia telangiectasia was compared with that of cultures from normal subjects. The results confirm previous observations of an association between ataxia telangiectasia and enhanced in vitro radiosensitivity, and also suggest that clonal survival of x-irradiated cultures of skin fibroblasts is a valuable technique for confirming the diagnosis of the disease.

MeSH Terms

Adolescent Ataxia Telangiectasia/diagnosis Cell Survival/radiation effects Cells, Cultured Child Child, Preschool Dose-Response Relationship, Radiation Evaluation Studies as Topic Female Fibroblasts/radiation effects Humans Male Radiation Tolerance Skin/radiation effects X-Rays

Authors & Affiliations

3 authors, click to expand affiliations / ORCID

Cox R

Hosking G P

Wilson J

References (15)

15 references, click to expand

Skin fibroblasts from a D-deletion type retinoblastoma patient are abnormally X-ray sensitive.
Nature. 1977 Apr 21;266(5604):726-7 PMID: 876352
Radiosensitivity in ataxia-telangiectasia.
Br J Radiol. 1975 May;48(569):374-6 PMID: 1139093
Growth of cultured cells from patients with ataxia-telangiectasia.
J Cell Physiol. 1976 NOV;89(3):429-31 PMID: 977661
Xeroderma pigmentosum: variants with normal DNA repair and normal sensitivity to ultraviolet light.
J Invest Dermatol. 1972 Mar;58(3):124-8 PMID: 5013606
Radiation reaction in ataxia telangiectasia.
Am J Dis Child. 1968 Nov;116(5):557-8 PMID: 5687489
Ataxia telangiectasia. Neoplasia, untoward response to x-irradiation, and tuberous sclerosis.
Am J Dis Child. 1967 Dec;114(6):617-25 PMID: 6072741
Serum-alpha-fetoprotein levels in patients with ataxia-telangiectasia.
Lancet. 1972 Nov 25;2(7787):1112-5 PMID: 4117204
Ataxia-telangiectasia: some historic, clinical and pathologic observations.
Birth Defects Orig Artic Ser. 1975;11(1):255-70 PMID: 1096982
Letter: Phenotypic manifestations of ataxia-telangiectasia.
Lancet. 1975 Nov 22;2(7943):1048 PMID: 53540
Letter: Changes in radiosensitivity during the in vitro growth of diploid human fibroblasts.
Int J Radiat Biol Relat Stud Phys Chem Med. 1974 Aug;26(2):193-6 PMID: 4547444
Somatic cell mutation. Detection and quantification of x-ray-induced mutation in cultured, diploid human fibroblasts.
Mutat Res. 1973 May;18(2):199-224 PMID: 4711931
Defective excision repair of gamma-ray-damaged DNA in human (ataxia telangiectasia) fibroblasts.
Nature. 1976 Apr 1;260(5550):444-7 PMID: 1256588
Is chromatid-type damage in ataxia telangiectasia after irradiation at G0 a consequence of defective repair?
Nature. 1976 Apr 1;260(5550):441-3 PMID: 1256587
Ataxia telangiectasia: a human mutation with abnormal radiation sensitivity.
Nature. 1975 Dec 4;258(5534):427-9 PMID: 1196376
In vitro radiosensitivity of human dipliod fibroblasts derived from patients with unusual clinical responses to radiation.
Radiology. 1976 Nov;121(2):479-82 PMID: 981631

Article Info

Journal

Archives of disease in childhood

Abbr.

Arch Dis Child

ISSN

1468-2044

DOI

10.1136/adc.53.5.386

Published

1978-05-00

Pages

386-90

Language

English

Region

England

NLM ID

0372434

PMCID

PMC1544929

Subset

External Links

PubMed source DOI: 10.1136/adc.53.5.386 PMC Full Text View journal details

Analysis Services

Ataxia telangiectasia. Evaluation of radiosensitivity in cultured skin fibroblasts as a diagnostic test.

Contact

WeChat Official Account