Role of progerin-induced telomere dysfunction in HGPS …
2010年8月1日 · We demonstrate that telomerase extends HGPS cellular lifespan by decreasing progerin-induced DNA-damage signaling and activation of p53 and Rb pathways that otherwise mediate the onset of premature senescence.
Hutchinson-Gilford Progeria Syndrome: Cellular Mechanisms and ...
2023年7月1日 · In this review, we present an updated overview of the biology of HGPS, including its clinical features, description of key cellular processes affected by progerin (nuclear morphology and function, nucleolar activity, mitochondrial function, protein nucleocytoplasmic trafficking and telomere homeostasis), as well as discussion of the therapeutic ...
Inhibition of DNA damage response at telomeres improves the ... - Nature
2019年11月18日 · Here we show that progerin-induced telomere dysfunction induces the transcription of tncRNAs. Their functional inhibition by sequence-specific telomeric antisense oligonucleotides (tASOs)...
Telomere length in Hutchinson-Gilford Progeria Syndrome
2009年6月1日 · In order to better understand the role of telomeres in HGPS, we examined the telomere length in cells from HGPS patients using two approaches. First, to determine how telomere lengths of individual chromosomes vary in fibroblasts of HGPS patients, we quantified telomere lengths using quantitative fluorescence in situ hybridization (Q-FISH ...
Telomerase therapy reverses vascular senescence and extends ... - PubMed
2021年11月7日 · Telomerase mRNA (hTERT) was used to treat HGPS ECs. Endothelial morphology and functions were assessed, as well as proteomic and transcriptional profiles with attention to inflammatory markers, DNA damage, and EC identity genes.
Progerin impairs 3D genome organization and induces fragile telomeres ...
2021年6月23日 · Remarkably, increasing dNTP levels in HGPS cells rescued fragile telomeres, and improved the replicative capacity of the cells. Our work highlights a functional connection between NE...
Role of progerin-induced telomere dysfunction in HGPS
2010年8月1日 · We demonstrate that telomerase extends HGPS cellular lifespan by decreasing progerin-induced DNA-damage signaling and activation of p53 and Rb pathways that otherwise mediate the onset of premature senescence.
Transient introduction of human telomerase mRNA improves …
2019年5月31日 · In HGPS cell lines characterized by short telomeres, transient transfections with hTERT mRNA increase telomere length, increase expression of telomere‐associated proteins, increase proliferative capacity and cellular lifespan, and reverse manifestations of cellular senescence as assessed by β‐galactosidase expression and secretion of ...
Progerin, damaged telomeres and aging - The Company of …
2010年8月1日 · Stuart Aaronson and colleagues have been investigating this puzzle and, on page 2605, they report that telomerase extends HGPS cellular lifespan by decreasing progerin-induced DNA-damage signalling, and the activation of the p53 and Rb pathways that mediate the premature senescence of HGPS cells.
Molecular insights into the premature aging disease progeria
In this review, we summarize the genetic cause of HGPS and consequences for posttranslational lamin processing and lamin functions. We also describe potential causative disease mechanisms and how they may contribute to the cellular, tissue and organismal phenotypes. Finally, we briefly summarize potential strategies for treatment of HGPS.