学科主题基础医学
A proteomic study of Hutchinson-Gilford progeria syndrome: Application of 2D-chromotography in a premature aging disease
Wang, Li2,3; Yang, Wu2,3; Ju, Weina1; Wang, Peirong2,3; Zhao, Xinliang2; Jenkins, Edmund C.1; Brown, W. Ted1; Zhong, Nanbert1,2,3
关键词Hutchinson-Gilford progeria syndrome ProteomeLab (TM) PF2D Protein Fractionation System Mass spectrum analysis Cytoskeleton proteins Intracellular calcium Cell cycle
刊名BIOCHEMICAL AND BIOPHYSICAL RESEARCH COMMUNICATIONS
2012-01-27
DOI10.1016/j.bbrc.2011.12.056
417期:4页:1119-1126
收录类别SCI
文章类型Article
WOS标题词Science & Technology
类目[WOS]Biochemistry & Molecular Biology ; Biophysics
研究领域[WOS]Biochemistry & Molecular Biology ; Biophysics
关键词[WOS]LAMIN-A ; HUMAN FIBROBLASTS ; NUCLEAR-ENVELOPE ; IN-VITRO ; EMERIN ; A/C ; LAMINOPATHIES ; EXPRESSION ; ACTIN ; IDENTIFICATION
英文摘要

The Hutchinson-Gilford progeria syndrome (HGPS) is a rare genetic disease characterized by segmental premature aging. Applying a two-dimensional chromatographic proteomic approach, the 20 Protein Fractionation System (PF2D), we identified 30 differentially expressed proteins in cultured HGPS fibroblasts. We categorized them into five groups: methylation, calcium ion binding, cytoskeleton, duplication, and regulation of apoptosis. Among these 30 proteins, 23 were down-regulated, while seven were up-regulated in HGPS fibroblasts as compared to normal fibroblasts. Three differentially expressed cytoskeleton proteins, vimentin, actin, and tubulin, were validated via Western blotting and characterized by immunostaining that revealed densely thickened bundles and irregular structures. Furthermore in the HGPS cells, the cell cycle G1 phase was elongated and the concentration of free cytosolic calcium was increased, suggesting intracellular retention of calcium. The results that we obtained have implications for understanding the aging process. (C) 2011 Elsevier Inc. All rights reserved.

语种英语
WOS记录号WOS:000300196100003
项目编号985-2-035-39 ; 2007CB511902 ; 30671157
资助机构Chinese Ministry of Education ; Ministry of Science and Technology ; National Nature Science Foundation ; New York State Office for People with Developmental Disabilities (NYS OPWDD)
引用统计
被引频次:9[WOS]   [WOS记录]     [WOS相关记录]
文献类型期刊论文
版本出版稿
条目标识符http://ir.bjmu.edu.cn/handle/400002259/55794
专题北京大学基础医学院_北京大学医学遗传中心
作者单位1.Peking Univ, Ctr Med Genet, Beijing 100871, Peoples R China
2.New York State Inst Basic Res Dev Disabil, Dept Human Genet, Staten Isl, NY 10314 USA
3.Peking Univ, Hlth Sci Ctr, Dept Med Genet, Beijing 100871, Peoples R China
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Wang, Li,Yang, Wu,Ju, Weina,et al. A proteomic study of Hutchinson-Gilford progeria syndrome: Application of 2D-chromotography in a premature aging disease[J]. BIOCHEMICAL AND BIOPHYSICAL RESEARCH COMMUNICATIONS,2012,417(4):1119-1126.
APA Wang, Li.,Yang, Wu.,Ju, Weina.,Wang, Peirong.,Zhao, Xinliang.,...&Zhong, Nanbert.(2012).A proteomic study of Hutchinson-Gilford progeria syndrome: Application of 2D-chromotography in a premature aging disease.BIOCHEMICAL AND BIOPHYSICAL RESEARCH COMMUNICATIONS,417(4),1119-1126.
MLA Wang, Li,et al."A proteomic study of Hutchinson-Gilford progeria syndrome: Application of 2D-chromotography in a premature aging disease".BIOCHEMICAL AND BIOPHYSICAL RESEARCH COMMUNICATIONS 417.4(2012):1119-1126.
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