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Differential stem cell aging kinetics in Hutchinson-Gilford progeria syndrome and Werner syndrome

文献类型：期刊论文

作者Wu, Zeming2,3,4; Qu, Jing2,4; Zhang, Weiqi3,4; Wang, Wei3,4; Chen, Chang3,4; Liu, Guang-Hui3,4,5; Song, Moshi4; Zhang, Weiqi5; Li, Wei5; Lei, Jinghui5
刊名PROTEIN & CELL
出版日期2018
卷号9期号:4页码:333-350
关键词WRN lamin HGPS Werner syndrome stem cell aging
ISSN号1674-800X
DOI10.1007/s13238-018-0517-8
文献子类Article
英文摘要Hutchinson-Gilford progeria syndrome (HGPS) and Werner syndrome (WS) are two of the best characterized human progeroid syndromes. HGPS is caused by a point mutation in lamin A (LMNA) gene, resulting in the production of a truncated protein product-progerin. WS is caused by mutations in WRN gene, encoding a loss-of-function RecQ DNA helicase. Here, by gene editing we created isogenic human embryonic stem cells (ESCs) with heterozygous (G608G/+) or homozygous (G608G/G608G) LMNA mutation and biallelic WRN knockout, for modeling HGPS and WS pathogenesis, respectively. While ESCs and endothelial cells (ECs) did not present any features of premature senescence, HGPS- and WS-mesenchymal stem cells (MSCs) showed aging-associated phenotypes with different kinetics. WS-MSCs had early-onset mild premature aging phenotypes while HGPS-MSCs exhibited late-onset acute premature aging characterisitcs. Taken together, our study compares and contrasts the distinct pathologies underpinning the two premature aging disorders, and provides reliable stem-cell based models to identify new therapeutic strategies for pathological and physiological aging.
学科主题Cell Biology
WOS关键词TARGETED GENE CORRECTION ; DNA-DAMAGE ACCUMULATION ; GENOMIC INSTABILITY ; HUMAN FIBROBLASTS ; DISEASE ; LAMIN ; MODEL ; SENESCENCE ; DEFECTS ; IPSCS
语种英语
WOS记录号WOS:000428849800003
出版者SPRINGEROPEN
版本出版稿
源URL[http://202.127.25.144/handle/331004/990]  
专题中国科学院上海生命科学研究院营养科学研究所
作者单位1.Jinan Univ, Inst Aging & Regenerat Med, Minist Educ, Key Lab Regenerat Med, Guangzhou 510632, Guangdong, Peoples R China,
2.Chinese Acad Sci, Inst Zool, State Key Lab Stem Cell & Reprod Biol, Beijing 100101, Peoples R China;
3.Chinese Acad Sci, Inst Biophys, CAS Ctr Excellence Biomacromol, Natl Lab Biomacromol, Beijing 100101, Peoples R China;
4.Univ Chinese Acad Sci, Beijing 100049, Peoples R China;
5.Capital Med Univ, Xuanwu Hosp, Natl Clin Res Ctr Geriatr Disorders, Beijing 100053, Peoples R China;
6.Chinese Acad Sci, Inst Zool, State Key Lab Biomembrane & Membrane Biotechnol, Beijing 100101, Peoples R China;
7.Chinese Acad Sci, Shanghai Inst Biol Sci, CAS MPG Partner Inst Computat Biol, Key Lab Computat Biol, Shanghai 200031, Peoples R China;
8.Peking Univ, Hlth Sci Ctr, Sch Basic Med Sci, Dept Med Genet, Beijing 100191, Peoples R China;
9.Capital Med Univ, Beijing Childrens Hosp, Natl Ctr Childrens Hlth, Dept Pediat Endocrinol & Genet Metab, Beijing 100045, Peoples R China;
10.Osaka Univ, Inst Adv Cocreat Studies, Osaka 5608531, Japan;
推荐引用方式
GB/T 7714		 Wu, Zeming,Qu, Jing,Zhang, Weiqi,et al. Differential stem cell aging kinetics in Hutchinson-Gilford progeria syndrome and Werner syndrome[J]. PROTEIN & CELL,2018,9(4):333-350.
APA Wu, Zeming.,Qu, Jing.,Zhang, Weiqi.,Wang, Wei.,Chen, Chang.,...&,.(2018).Differential stem cell aging kinetics in Hutchinson-Gilford progeria syndrome and Werner syndrome.PROTEIN & CELL,9(4),333-350.
MLA Wu, Zeming,et al."Differential stem cell aging kinetics in Hutchinson-Gilford progeria syndrome and Werner syndrome".PROTEIN & CELL 9.4(2018):333-350.

入库方式： OAI收割

来源：上海营养与健康研究所

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