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用扫描质子微探针进行成纤维细胞的元素微量分析及其在门克斯病中的应用。

Elemental microanalysis of fibroblasts by a scanning proton microprobe and application to Menkes' disease.

作者信息

Allan G L, Camakaris J, Legge G J

机构信息

Micro Analytical Research Centre, School of Physics, University of Melbourne, Parkville, Victoria, Australia.

出版信息

Biol Trace Elem Res. 1994 Feb;40(2):103-26. doi: 10.1007/BF02950785.

DOI:10.1007/BF02950785
PMID:7514017
Abstract

A scanning proton microprobe has been used for the elemental microanalysis of individual fibroblast cells. Both normal fibroblasts and fibroblasts cultured from patients with Menkes' disease, an X-linked genetic disorder known to be associated with defective copper metabolism, were examined by the probe. The cells were cultured on a thin ultra-clean nylon foil and retained on that surface for analysis. The focused high-energy proton beam was used to irradiate selected individual cells and elemental information was derived from X-ray and backscattered proton data. The sensitivity of the scanning proton microprobe to trace concentrations of heavy elements has allowed this elemental information to be used to identify individual cells as being either normal or a Menkes' mutant. The cell identification was based on the application of discriminate analysis to a data set formed from the ratios of copper to each of the macroelements present in the cell. This method of cell identification offers the promise of rapid diagnosis of Menkes' disease.

摘要

扫描质子微探针已用于单个成纤维细胞的元素微分析。该探针检测了正常成纤维细胞以及从患有门克斯病(一种已知与铜代谢缺陷相关的X连锁遗传病)患者身上培养的成纤维细胞。细胞在超薄超净尼龙箔上培养,并保留在该表面进行分析。聚焦的高能质子束用于照射选定的单个细胞,元素信息来自X射线和背散射质子数据。扫描质子微探针对痕量重金属元素浓度的敏感性使得该元素信息可用于识别单个细胞是正常细胞还是门克斯突变细胞。细胞识别基于对由细胞中铜与每种大量元素的比率形成的数据集应用判别分析。这种细胞识别方法有望实现门克斯病的快速诊断。

相似文献

1
Elemental microanalysis of fibroblasts by a scanning proton microprobe and application to Menkes' disease.用扫描质子微探针进行成纤维细胞的元素微量分析及其在门克斯病中的应用。
Biol Trace Elem Res. 1994 Feb;40(2):103-26. doi: 10.1007/BF02950785.
2
Effect of copper on Menkes' and normal cultured skin fibroblasts.铜对门克斯病及正常培养的皮肤成纤维细胞的影响。
Dev Pharmacol Ther. 1980;1(5):305-17.
3
Metallothionein in Menkes' disease: induction in cultured muscle cells.
J Neurol Sci. 1990 Dec;100(1-2):50-6. doi: 10.1016/0022-510x(90)90012-c.
4
[Copper level and metallothionein-like Cu-binding protein in cultured skin fibroblasts from patients with Menkes' disease and Wilson's disease].[门克斯病和威尔逊病患者培养的皮肤成纤维细胞中的铜水平及金属硫蛋白样铜结合蛋白]
No To Shinkei. 1984 Nov;36(11):1063-8.
5
Effect of medium copper concentration on the growth, uptake and intracellular balance of copper and zinc in Menkes' and normal control cells.中等铜浓度对门克斯病细胞和正常对照细胞中铜和锌的生长、摄取及细胞内平衡的影响。
Biometals. 1994 Jul;7(3):253-60. doi: 10.1007/BF00149556.
6
Abnormal copper metabolism and regulation of metallothionein gene expression in Menkes' disease.门克斯病中铜代谢异常及金属硫蛋白基因表达调控
Experientia Suppl. 1987;52:477-80. doi: 10.1007/978-3-0348-6784-9_48.
7
Menkes' disease: abnormal metallothionein gene regulation in response to copper.门克斯病:响应铜的异常金属硫蛋白基因调控。
Cell. 1985 Feb;40(2):301-9. doi: 10.1016/0092-8674(85)90144-8.
8
Metallothionein accumulation may account for intracellular copper retention in Menkes' disease.金属硫蛋白的蓄积可能是门克斯病细胞内铜潴留的原因。
J Biol Chem. 1982 Apr 25;257(8):4639-45.
9
Synthesis of a metallothionein-like protein in cultured human skin fibroblasts: relation to abnormal copper distribution in Menkes' disease.培养的人皮肤成纤维细胞中金属硫蛋白样蛋白的合成:与门克斯病中铜分布异常的关系。
J Cell Physiol. 1981 Mar;106(3):339-48. doi: 10.1002/jcp.1041060303.
10
Reduced lysyl oxidase activity in skin fibroblasts from patients with Menkes' syndrome.门克斯综合征患者皮肤成纤维细胞中赖氨酰氧化酶活性降低。
Biochem J. 1980 Nov 15;192(2):579-86. doi: 10.1042/bj1920579.

本文引用的文献

1
Altered copper metabolism in cultured cells from human Menkes' syndrome and mottled mouse mutants.来自人类门克斯综合征和斑驳小鼠突变体的培养细胞中铜代谢的改变。
Biochem Genet. 1980 Feb;18(1-2):117-31. doi: 10.1007/BF00504364.
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Mutations affecting trace elements in humans and animals. A genetic approach to an understanding of trace elements.影响人类和动物体内微量元素的突变。一种理解微量元素的遗传学方法。
Adv Hum Genet. 1983;13:149-216. doi: 10.1007/978-1-4615-8342-4_4.
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Preparation of individual human diploid fibroblasts and study of ion transport.
人二倍体成纤维细胞的制备及离子转运研究。
Am J Physiol. 1985 Jan;248(1 Pt 1):C154-64. doi: 10.1152/ajpcell.1985.248.1.C154.
4
Proton microscopy and microanalysis--biological applications.质子显微镜术与微分析——生物学应用
Ultramicroscopy. 1988;24(2-3):283-97. doi: 10.1016/0304-3991(88)90316-6.
5
Menkes disease: a biochemical abnormality in cultured human fibroblasts.门克斯病:培养的人成纤维细胞中的一种生化异常。
Proc Natl Acad Sci U S A. 1976 Feb;73(2):604-6. doi: 10.1073/pnas.73.2.604.