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来自正常、着色性干皮病和毛发硫营养不良供体的未刺激和刺激的T淋巴细胞及成纤维细胞对UV-C致死作用的反应比较。

A comparison of the response of unstimulated and stimulated T-lymphocytes and fibroblasts from normal, xeroderma pigmentosum and trichothiodystrophy donors to the lethal action of UV-C.

作者信息

Arlett C F, Harcourt S A, Cole J, Green M H, Anstey A V

机构信息

MRC Cell Mutation Unit, University of Sussex, Falmer, Brighton, Great Britain.

出版信息

Mutat Res. 1992 Mar;273(2):127-35. doi: 10.1016/0921-8777(92)90074-d.

DOI:10.1016/0921-8777(92)90074-d
PMID:1372096
Abstract

Unstimulated T-lymphocytes from normal donors are significantly more sensitive to the lethal effects of UV-C than either stimulated T-lymphocytes or fibroblasts as judged by colony-forming ability. Data from other studies suggest that excision repair is more effective in stimulated than unstimulated T-lymphocytes leading to the prediction that these differences in survival should be minimal in cells established from excision defective donors. The prediction was met with XP6BR, a donor of unknown complementation group. For 3 XP's from complementation group D, however, enhanced survival in stimulated T-cells was observed. With cells from an excision-defective TTD who was included in complementation group D of XP both fibroblasts and unstimulated T-lymphocytes were hypersensitive. For a second excision defective TTD patient who was excluded from complementation group D, the unstimulated T-lymphocytes were more resistant than those of normal donors although the fibroblasts were hypersensitive. These results suggest that the in vitro response of stimulated T-lymphocytes or fibroblasts may not reflect the in vivo response of cells, as measured by the response of unstimulated T-lymphocytes.

摘要

根据集落形成能力判断,来自正常供体的未受刺激的T淋巴细胞对UV-C的致死效应比受刺激的T淋巴细胞或成纤维细胞更敏感。其他研究的数据表明,切除修复在受刺激的T淋巴细胞中比在未受刺激的T淋巴细胞中更有效,这导致预测这些生存差异在来自切除缺陷供体的细胞中应最小。这一预测在XP6BR(一个互补组未知的供体)中得到了验证。然而,对于来自互补组D的3名XP患者,观察到受刺激的T细胞存活率提高。对于一名被纳入XP互补组D的切除缺陷型TTD患者的细胞,成纤维细胞和未受刺激的T淋巴细胞都高度敏感。对于另一名被排除在互补组D之外的切除缺陷型TTD患者,未受刺激的T淋巴细胞比正常供体的更具抗性,尽管成纤维细胞高度敏感。这些结果表明,受刺激的T淋巴细胞或成纤维细胞的体外反应可能无法反映未受刺激的T淋巴细胞反应所衡量的细胞体内反应。

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A comparison of the response of unstimulated and stimulated T-lymphocytes and fibroblasts from normal, xeroderma pigmentosum and trichothiodystrophy donors to the lethal action of UV-C.来自正常、着色性干皮病和毛发硫营养不良供体的未刺激和刺激的T淋巴细胞及成纤维细胞对UV-C致死作用的反应比较。
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Xeroderma pigmentosum (complementation group D) mutation is present in patients affected by trichothiodystrophy with photosensitivity.患有光敏感型毛发硫营养不良的患者存在着色性干皮病(互补组D)突变。
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引用本文的文献

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Xeroderma pigmentosum and trichothiodystrophy are associated with different mutations in the XPD (ERCC2) repair/transcription gene.着色性干皮病和毛发硫营养不良与XPD(ERCC2)修复/转录基因中的不同突变相关。
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Xeroderma pigmentosum variant: 5 years of tumour suppression by etretinate.
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