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结节性硬化症患者基质细胞的异常基因表达。

Abnormal gene expressions of stroma cells in patients with tuberous sclerosis.

作者信息

Ishibashi Y, Watanabe R, Nogita T, Takahashi T, Onodera K, Kimura G

机构信息

Department of Dermatology, Faculty of Medicine, University of Tokyo, Japan.

出版信息

Ann N Y Acad Sci. 1991;615:228-42. doi: 10.1111/j.1749-6632.1991.tb37764.x.

DOI:10.1111/j.1749-6632.1991.tb37764.x
PMID:2039147
Abstract

The characteristics of stroma cells in tissues and the cloned cells, which derived from adenomata sebacea in the skin of patients with tuberous sclerosis, were investigated using histochemical, biochemical, flow cytofluorometric, electron microscopic, and immunofluorescent techniques, and the following results were obtained: 1. The stroma cells from adenoma sebaceum show a distinct pleomorphism. 2. They reveal glial cell-like gene expressions rather than fibroblastic ones in both tissue and cultured condition. 3. In culture their DNA histograms show abnormal patterns, suggesting the presence of nuclei that contain unequal quantities of DNA. 4. Some of these cells show distinct chromosome disarrangement in metaphase followed by abnormal divisions indicating the presence of a functional defect in the "centromere-microtubule-centriole" system. 5. It is characteristic that even in the cloned cells some changes spontaneously occur in the growth and differentiation of cells with an unstable frequency.

摘要

利用组织化学、生物化学、流式细胞荧光测定法、电子显微镜及免疫荧光技术,对结节性硬化症患者皮肤皮脂腺腺瘤中的基质细胞和克隆细胞的特性进行了研究,获得了以下结果:1. 皮脂腺瘤的基质细胞呈现出明显的多形性。2. 在组织和培养条件下,它们表现出神经胶质样基因表达,而非成纤维细胞样基因表达。3. 在培养中,它们的DNA直方图显示出异常模式,表明存在含有不等量DNA的细胞核。4. 其中一些细胞在中期显示出明显的染色体紊乱,随后出现异常分裂,表明“着丝粒 - 微管 - 中心粒”系统存在功能缺陷。5. 其特点是,即使在克隆细胞中,细胞的生长和分化也会以不稳定的频率自发发生一些变化。

相似文献

1
Abnormal gene expressions of stroma cells in patients with tuberous sclerosis.结节性硬化症患者基质细胞的异常基因表达。
Ann N Y Acad Sci. 1991;615:228-42. doi: 10.1111/j.1749-6632.1991.tb37764.x.
2
Abnormal DNA histograms of stroma cells in patients with tuberous sclerosis. A cytofluorometric study.结节性硬化症患者基质细胞的异常DNA直方图。一项细胞荧光分析研究。
J Dermatol. 1984 Feb;11(1):1-6. doi: 10.1111/j.1346-8138.1984.tb01432.x.
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Distribution of glial fibrillary acidic protein (GFAP) in the intermediate filaments of the cultured cells from a patient with tuberous sclerosis.胶质纤维酸性蛋白(GFAP)在结节性硬化症患者培养细胞中间丝中的分布。
J Dermatol. 1990 Jul;17(7):395-402. doi: 10.1111/j.1346-8138.1990.tb01665.x.
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Abnormal division and gene expression in cultured cells from a patient with tuberous sclerosis.结节性硬化症患者培养细胞中的异常分裂和基因表达。
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[The so-called sebaceous adenoma in the Bourneville-Pringle syndrome].
Z Haut Geschlechtskr. 1973 Mar 1;48(5):181-6.
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Abnormal and unstable patterns of the DNA of stroma cells in a patient with tuberous sclerosis--a flow cytofluorometric investigation.结节性硬化症患者基质细胞DNA的异常与不稳定模式——流式细胞荧光分析研究
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Tuberose sclerosis without adenoma sebaceum.无皮脂腺瘤的结节性硬化症。
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9
Familial cylindromatosis mimicking tuberous sclerosis complex and confirmation of the cylindromatosis locus, CYLD1, in a large family.疑似结节性硬化症的家族性圆柱瘤病及在一个大家族中对圆柱瘤病基因座CYLD1的确认
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Biochemical study of cells cultured from a patient with tuberous sclerosis.对结节性硬化症患者培养的细胞进行的生化研究。
J Dermatol. 1992 Nov;19(11):909-13. doi: 10.1111/j.1346-8138.1992.tb03804.x.

引用本文的文献

1
Familial cylindromatosis mimicking tuberous sclerosis complex and confirmation of the cylindromatosis locus, CYLD1, in a large family.疑似结节性硬化症的家族性圆柱瘤病及在一个大家族中对圆柱瘤病基因座CYLD1的确认
J Med Genet. 1998 Oct;35(10):841-5. doi: 10.1136/jmg.35.10.841.