دورية أكاديمية
A proteomic study of Hutchinson-Gilford progeria syndrome: Application of 2D-chromotography in a premature aging disease
| العنوان: | A proteomic study of Hutchinson-Gilford progeria syndrome: Application of 2D-chromotography in a premature aging disease |
|---|---|
| المؤلفون: | Wang, Li, Yang, Wu, Ju, Weina, Wang, Peirong, Zhao, Xinliang, Jenkins, Edmund C., Brown, W. Ted, Zhong, Nanbert |
| المساهمون: | Zhong, N (reprint author), New York State Inst Basic Res Dev Disabil, Dept Human Genet, 1050 Forest Hill Rd, Staten Isl, NY 10314 USA., New York State Inst Basic Res Dev Disabil, Dept Human Genet, Staten Isl, NY 10314 USA., Peking Univ, Ctr Med Genet, Beijing 100871, Peoples R China., Peking Univ, Hlth Sci Ctr, Dept Med Genet, Beijing 100871, Peoples R China., New York State Inst Basic Res Dev Disabil, Dept Human Genet, 1050 Forest Hill Rd, Staten Isl, NY 10314 USA. |
| المصدر: | PubMed ; SCI |
| بيانات النشر: | 生物化学与生物物理学研究通讯 |
| سنة النشر: | 2012 |
| المجموعة: | Peking University Institutional Repository (PKU IR) / 北京大学机构知识库 |
| مصطلحات موضوعية: | Hutchinson-Gilford progeria syndrome, ProteomeLab (TM) PF2D Protein Fractionation System, Mass spectrum analysis, Cytoskeleton proteins, Intracellular calcium, Cell cycle, LAMIN-A, HUMAN FIBROBLASTS, NUCLEAR-ENVELOPE, IN-VITRO, EMERIN, A/C, LAMINOPATHIES, EXPRESSION, ACTIN, IDENTIFICATION |
| الوصف: | 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. ; http://gateway.webofknowledge.com/gateway/Gateway.cgi?GWVersion=2&SrcApp=PARTNER_APP&SrcAuth=LinksAMR&KeyUT=WOS:000300196100003&DestLinkType=FullRecord&DestApp=ALL_WOS&UsrCustomerID=8e1609b174ce4e31116a60747a720701Test ; Biochemistry & Molecular Biology ; Biophysics ; SCI(E) ; PubMed ; 7 ; ARTICLE ; 4 ; 1119-1126 ; 417 |
| نوع الوثيقة: | journal/newspaper |
| اللغة: | English |
| تدمد: | 0006-291X 22210539 |
| العلاقة: | BIOCHEMICAL AND BIOPHYSICAL RESEARCH COMMUNICATIONS.2012,417,(4),1119-1126.; 661301; http://hdl.handle.net/20.500.11897/192617Test; WOS:000300196100003 |
| DOI: | 10.1016/j.bbrc.2011.12.056 |
| الإتاحة: | https://doi.org/20.500.11897/192617Test https://doi.org/10.1016/j.bbrc.2011.12.056Test https://hdl.handle.net/20.500.11897/192617Test |
| رقم الانضمام: | edsbas.9857A3F2 |
| قاعدة البيانات: | BASE |
| تدمد: | 0006291X 22210539 |
|---|---|
| DOI: | 10.1016/j.bbrc.2011.12.056 |