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E-GEOD-24487 - Recapitulation of human premature aging by using iPSCs from Hutchinson-Gilford progeria syndrome

Released on 14 March 2011, last updated on 2 May 2014
Homo sapiens
Samples (10)
Array (1)
Protocols (9)
Hutchinson-Gilford progeria syndrome (HGPS) is a rare and fatal human premature aging disease1-5, characterized by premature atherosclerosis and degeneration of vascular smooth muscle cells (SMCs)6-8. HGPS is caused by a single-point mutation in the LMNA gene, resulting in the generation of progerin, a truncated mutant of lamin A. Accumulation of progerin leads to various aging-associated nuclear defects including disorganization of nuclear lamina and loss of heterochromatin9-12. Here, we report the generation of induced pluripotent stem cells (iPSCs) from fibroblasts obtained from patients with HGPS. HGPS-iPSCs show absence of progerin, and more importantly, lack the nuclear envelope and epigenetic alterations normally associated with premature aging. Upon differentiation of HGPS-iPSCs, progerin and its associated aging consequences are restored. In particular, directed differentiation of HGPS-iPSCs to SMCs leads to the appearance of premature senescent SMC phenotypes associated with vascular aging. Additionally, our studies identify DNA-dependent protein kinase catalytic subunit (DNAPKcs) as a component of the progerin-containing protein complex. The absence of nuclear DNAPKcs correlates with premature as well as physiological aging. Since progerin also accumulates during physiological aging6,12,13, our results provide an in vitro iPSC-based model with an acceleration progerin accumulation to study the pathogenesis of human premature and physiological vascular aging. Microarray gene expression profiling was done to: (1) Compare differences between WT fibroblasts and fibroblasts from patients suffering of the Hutchinson-Gilford progeria syndrome (2) Check that iPSC originating from WT and patients are in fact similar to ESC
Experiment type
transcription profiling by array 
Stephanie Boue <>, Athanasia D Panopoulos, Basam Barkho, Christopher Walsh, Dinh Diep, Guang-Hui Liu, Ho-Lim Fung, James Thompson, Jing Qu, John Yates III, Juan-Carlos Izpisua-Belmonte, Kun Zhang, Leo Kurian, Sergio Ruiz, Sheng-Lian Yang
Recapitulation of premature ageing with iPSCs from Hutchinson-Gilford progeria syndrome. Liu GH, Barkho BZ, Ruiz S, Diep D, Qu J, Yang SL, Panopoulos AD, Suzuki K, Kurian L, Walsh C, Thompson J, Boue S, Fung HL, Sancho-Martinez I, Zhang K, Yates J 3rd, Izpisua Belmonte JC. , PMID:21346760
Investigation descriptionE-GEOD-24487.idf.txt
Sample and data relationshipE-GEOD-24487.sdrf.txt
Raw data (1)
Processed data (1)
Array designA-AFFY-44.adf.txt
R ExpressionSetE-GEOD-24487.eSet.r