1986 Fiscal Year Final Research Report Summary
Classification of neurological disorders from the viewpoints of DNA instability
| Project/Area Number |
59480216
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| Research Category |
Grant-in-Aid for General Scientific Research (B)
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| Allocation Type | Single-year Grants |
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| Research Field |
Neurology
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| Research Institution | National Center of Neurology and Psychiatry (N C N P) (1986)
国立武蔵療養所 (1984-1985) |
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Principal Investigator |
ARIMA Masataka National Institute of Neuroscience,National Center of Neurology and Psychiatry, その他, その他 (10032179)
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| Co-Investigator(Kenkyū-buntansha) |
林 昭子 国立武蔵療養所神経センター, 疾病研究第二部, 研究員
佐藤 充 国立武蔵療養所神経センター, 疾病研究第二部, 研究員
YOSHIDA Yutaka ibid, 疾病研究第二部, 研究員
TANAKA Harumi National Institute of Neuroscience, National Center of Neurology and Psychiatry, 疾病研究第二部, 室長 (00032334)
HAYASHI Akiko ibid
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| Project Period (FY) |
1984 – 1986
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| Keywords | X-ray Seneitivity / UV-sensitivity / Cell survival / Colony forming test / Tuberous sclerosis / コロニー形成試験 / 神経線維腫症 |
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| Research Abstract |
Cultured cell strains from patients with ataxia-telangiectasia, Cockayne syndrome, normal and tumorous skin of tuberous sclerosis or meurofibromatosis patients, and patients with other neurological diseases were examined for their sensitivity to X-rays and/or UV as determined from their colony-forming ability.
All cell strains except one showed hypersensitivity to Xrays in ataxia-telangiectasia. All strains derived from normal-appearing skin of patients with tuberous sclerosis or neurofibromatosis showed sensitivity within the normal control range. The mean <D_0> or <D_(10)> values of the strains from tumorous skin of patients tended to be lower compared to those for normal skin. Cells from Wilson's disease and Cockayne syndrome patients showed sensitivity to X-rays within normal range. Kinetic analysis of the rate of rejoining of DNA single strand breaks after X-irradiation indicated that the rate of rejoining involved at least two components, an initial fast component and a slower component. Tuberous sclerosis strains were proficient as to DNA single strand breaks, but they showed an increased rate of rejoining in the initial fast repair process as compared to normal controls.
Nine cell strains from Cockayne syndrome patients showed UV-sensitivity between that of xeroderma pigmentosum type A and normal controls. We found another cell strain of Cockayne syndrome with normal UV survival but delayed recovery of RNA synthesis after UV irradiation. These results indicate that combination of tests for UV-sensitivity may be necessary for the diagnosis of Cockayne syndrome in some occasion.
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