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共济失调毛细血管扩张症患者皮肤成纤维细胞系中DNA聚合酶β的测定。

Measurement of DNA polymerase beta in skin fibroblast cell lines from patients with ataxia telangiectasia.

作者信息

Mitchell J B, Karawya E, Kinsella T J, Wilson S H

出版信息

Mutat Res. 1985 Nov;146(3):295-300. doi: 10.1016/0167-8817(85)90071-9.

DOI:10.1016/0167-8817(85)90071-9
PMID:4058446
Abstract

DNA polymerase beta levels were measured in 4 cell lines of normal human skin fibroblasts and in 5 cell lines of skin fibroblasts from patients with ataxia telangiectasia, an autosomal recessive disease exhibiting marked X-ray sensitivity. The enzyme specific activities for the normal lines were similar and the mean value was 2-fold lower than the mean value for the ataxia lines. With both kinds of cells, the enzyme level did not change as the cultures progressed from logarithmic to stationary phase of growth. Thus, this putative DNA repair enzyme appears to be 'constitutive' in human skin fibroblast lines, and a modest elevation of beta-polymerase activity is associated with ataxia telangiectasia. These results are discussed in the context to current views about DNA-repair enzymes in X-ray-sensitive cultured mammalian cells.

摘要

在4株正常人皮肤成纤维细胞系和5株共济失调毛细血管扩张症患者的皮肤成纤维细胞系中测量了DNA聚合酶β的水平。共济失调毛细血管扩张症是一种常染色体隐性疾病,表现出明显的X射线敏感性。正常细胞系的酶比活性相似,其平均值比共济失调细胞系的平均值低2倍。对于这两种细胞,随着培养物从对数生长期进入稳定生长期,酶水平没有变化。因此,这种假定的DNA修复酶在人皮肤成纤维细胞系中似乎是“组成型的”,并且β聚合酶活性的适度升高与共济失调毛细血管扩张症相关。结合当前关于X射线敏感的培养哺乳动物细胞中DNA修复酶的观点对这些结果进行了讨论。

相似文献

1
Measurement of DNA polymerase beta in skin fibroblast cell lines from patients with ataxia telangiectasia.共济失调毛细血管扩张症患者皮肤成纤维细胞系中DNA聚合酶β的测定。
Mutat Res. 1985 Nov;146(3):295-300. doi: 10.1016/0167-8817(85)90071-9.
2
An enzyme activity in normal and ataxia telangiectasia cell lines which is involved in the repair of gamma-irradiation-induced DNA damage.一种存在于正常细胞系和共济失调毛细血管扩张症细胞系中的酶活性,其参与γ射线诱导的DNA损伤修复。
Biochem J. 1980 Jun 15;188(3):677-82. doi: 10.1042/bj1880677.
3
DNA repair enzyme deficiency and in vitro complementation of the enzyme activity in cell-free extracts from ataxia telangiectasia fibroblasts.共济失调毛细血管扩张症成纤维细胞的DNA修复酶缺陷及无细胞提取物中酶活性的体外互补
Biochim Biophys Acta. 1981 Aug 27;655(1):49-53. doi: 10.1016/0005-2787(81)90065-4.
4
DNA repair enzymes in ataxia telangiectasia and Bloom's syndrome fibroblasts.共济失调毛细血管扩张症和布卢姆综合征成纤维细胞中的DNA修复酶
Biochim Biophys Acta. 1977 Dec 14;479(4):497-500. doi: 10.1016/0005-2787(77)90042-9.
5
Normal activity of nucleoside phosphorylase, superoxide dismutase and catalase in skin fibroblasts cultured from ataxia-telangiectasia patients.共济失调毛细血管扩张症患者皮肤成纤维细胞中核苷磷酸化酶、超氧化物歧化酶和过氧化氢酶的正常活性。
Isr J Med Sci. 1978 Feb;14(2):284-7.
6
Superoxide dismutase and catalase activities in Ataxia telangiectasia and normal fibroblast cell extracts.共济失调毛细血管扩张症与正常成纤维细胞提取物中的超氧化物歧化酶和过氧化氢酶活性。
Mutat Res. 1979 Jul;61(2):381-6. doi: 10.1016/0027-5107(79)90141-6.
7
An apparent correlation between the inhibition of induced ornithine decarboxylase activity by gamma radiation and the capacity for DNA repair synthesis in normal and ataxia telangiectasia human fibroblasts: no correlation with cell survival.γ射线对诱导型鸟氨酸脱羧酶活性的抑制与正常及共济失调毛细血管扩张症患者人成纤维细胞中DNA修复合成能力之间的明显相关性:与细胞存活无关。
Radiat Environ Biophys. 1981;20(1):21-8. doi: 10.1007/BF01323923.
8
DNA polymerases alpha beta and gamma in inherited diseases affecting DNA repair.影响DNA修复的遗传性疾病中的DNA聚合酶α、β和γ
Nucleic Acids Res. 1978 Jun;5(6):2189-96. doi: 10.1093/nar/5.6.2189.
9
Overproduction of topoisomerase II in an ataxia telangiectasia fibroblast cell line: comparison with a topoisomerase II-overproducing hamster cell mutant.共济失调毛细血管扩张症成纤维细胞系中拓扑异构酶II的过度产生:与拓扑异构酶II过度产生的仓鼠细胞突变体的比较。
Nucleic Acids Res. 1989 Feb 25;17(4):1337-51. doi: 10.1093/nar/17.4.1337.
10
Modulation of chromatin conformation by the histone deacetylase inhibitor trichostatin A promotes the removal of radiation-induced lesions in ataxia telangiectasia cell lines.组蛋白脱乙酰酶抑制剂曲古抑菌素A对染色质构象的调节促进共济失调毛细血管扩张症细胞系中辐射诱导损伤的清除。
Mutat Res Genet Toxicol Environ Mutagen. 2018 Dec;836(Pt A):109-116. doi: 10.1016/j.mrgentox.2018.06.016. Epub 2018 Jun 15.

引用本文的文献

1
The human DNA polymerase beta gene structure. Evidence of alternative splicing in gene expression.人类DNA聚合酶β基因结构。基因表达中选择性剪接的证据。
Nucleic Acids Res. 1994 Jul 25;22(14):2719-25. doi: 10.1093/nar/22.14.2719.
2
Ataxia-telangiectasia: an inherited disorder of ionizing-radiation sensitivity in man. Progress in the elucidation of the underlying biochemical defect.共济失调毛细血管扩张症:一种人类遗传性电离辐射敏感障碍。潜在生化缺陷阐释的进展。
Hum Genet. 1987 Mar;75(3):197-208. doi: 10.1007/BF00281059.
3
Structure of rat DNA polymerase beta revealed by partial amino acid sequencing and cDNA cloning.
Proc Natl Acad Sci U S A. 1986 Jul;83(14):5106-10. doi: 10.1073/pnas.83.14.5106.
4
Induction of beta-polymerase mRNA by DNA-damaging agents in Chinese hamster ovary cells.
Mol Cell Biol. 1989 Feb;9(2):851-3. doi: 10.1128/mcb.9.2.851-853.1989.
5
Characterization of DNA polymerase beta mRNA: cell-cycle and growth response in cultured human cells.DNA聚合酶β mRNA的特性:培养的人细胞中的细胞周期与生长反应
Nucleic Acids Res. 1988 Oct 25;16(20):9587-96. doi: 10.1093/nar/16.20.9587.
6
Protein binding elements in the human beta-polymerase promoter.人类β-聚合酶启动子中的蛋白质结合元件。
Nucleic Acids Res. 1990 Feb 25;18(4):919-28. doi: 10.1093/nar/18.4.919.