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Hutchinson-Gilford progeria syndrome (HGPS) is a premature aging disease caused by progerin accumulation. However, the mechanism of how progerin causes cell defects is unclear in HGPS cells. To further investigate the possible mechanism, we used Flag-tagged progerin as bait in mass spectrometry to s...
ORGANISM(S): Homo sapiens (Human) 
2023-01-30 | PXD039136 | Pride
Reprogramming somatic cells to induced pluripotent stem cells (iPSCs) sets their identity back to an embryonic age. This presents a fundamental hurdle for modeling late-onset disorders using iPSC-derived cells. We therefore developed a strategy to induce age-like features in multiple iPSC-derived li...
ORGANISM(S): Homo sapiens 
Disassembly of the nuclear envelope is an essential feature of mammalian cell division controlled by the phosphoryaltion of lamins via cyclin dependent kinases. This process is affected in cells expressing progerin, a lamin A allele found in patients with Hutchinson-Gilford Progeria syndrome. Proger...
ORGANISM(S): Homo sapiens 
Hutchinson-Gilford Progeria Syndrome (HGPS) is a rare, genetic premature aging disorder associated with severe atherosclerosis, often resulting in fatal heart attacks and strokes. Progerin, the mutant protein in HGPS, also is expressed in healthy individuals and may play a role in the development o...
ORGANISM(S): Homo sapiens 
HMC3-Progerin cells model for accelerated aging through inducible progerin expression
Analysis of p53-deficient (E6-expressing) human vascular smooth muscle cells (VSMCs) that express progerin, a mutated form of lamin A resposible for Hutchinson- Gilford progeria syndrome (HGPS). p53 pathway is associated with HGPS. Results provide insight into molecular mechanisms underlying vascula...
ORGANISM(S): Homo sapiens 
Hutchinson-Gilford Progeria Syndrome (HGPS) is caused by a point mutation in the LMNA gene that activates a cryptic donor splice site and yields a truncated form of prelamin A called progerin. Small amounts of progerin are also produced during normal aging. Studies with mouse models of HGPS have all...
ORGANISM(S): Mus musculus 
RNA-seq analysis after STXBP5 overexpression or STXBP5 knockdown in HA-progerin HEK293 cells
We compared the transcriptomes of isogenic diploid fibroblasts expressing progerin or elevated levels of wild-type prelamin A with that of wild-type fibroblasts. We subsequently used the reversion towards normal of two phenotypes, reduced cell growth and dismorphic nuclei, by treatment with farnesyl...
ORGANISM(S): Homo sapiens 
The premature aging disease Hutchinson-Gilford Progeria Syndrome (HGPS) is caused by constitutive production of progerin, a mutant form of the nuclear architectural protein lamin A1. Progerin is also sporadically expressed in wild type cells and has been linked to physiological aging. HGPS cells exh...
ORGANISM(S): Homo sapiens 
2008-02-09 | GSE10123 | GEO
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