【摘 要】
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Hutchinson-Gilford progeria syndrome (HGPS) and Wemer syndrome (WS) are two of the best characterized human progeroid syndromes.HGPS is caused by a point mutati
【机 构】
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State Key Laboratory of Stem Cell and Reproductive Biology, Institute of Zoology, Chinese Academy of
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Hutchinson-Gilford progeria syndrome (HGPS) and Wemer 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 gere 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 ram-cell based models to identify new therapeutic strategies for pathological and physiological aging.
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