Results 81 to 90 of about 2,732 (185)
Inhibiting farnesylation of progerin prevents the characteristic nuclear blebbing of Hutchinson-Gilford progeria syndrome [PDF]
, 2005 Hutchinson-Gilford progeria syndrome (HGPS) is a rare genetic disorder that is characterized by dramatic premature aging and accelerated cardiovascular disease.
Erdos, M. R. +9 more
core +2 more sources
Biology Open, 2012 Summary Hutchinson-Gilford progeria syndrome (HGPS, OMIM 176670) is a rare disorder characterized by segmental accelerated aging and early death from coronary artery disease or stroke. Nearly 90% of HGPS sufferers carry a G608G mutation within exon 11 of
Vera Wenzel +7 more
doaj +1 more source
MG132 Induces Progerin Clearance and Improves Disease Phenotypes in HGPS-like Patients’ Cells
Cells, 2022 Progeroid syndromes (PS), including Hutchinson-Gilford Progeria Syndrome (HGPS), are premature and accelerated aging diseases, characterized by clinical features mimicking physiological aging.
Karim Harhouri +9 more
doaj +1 more source
Cells, 2023 Hutchinson–Gilford Progeria Syndrome (HGPS) is an ultra-rare human premature aging disorder that precipitates death because of cardiac disease. Almost all cases of HGPS are caused by aberrant splicing of the LMNA gene that results in the production of a ...
So-mi Kang +9 more
doaj +1 more source
Journal of Cellular and Molecular Medicine, Volume 30, Issue 8, April 2026.ABSTRACT CRISPR‐Cas9 systems revolutionized gene editing, but inherent drawbacks, namely DNA double‐strand breaks (DSBs) and the difficulty of achieving precise repairs (due to low HDR efficiency), led researchers to invent new, more accurate gene editing tools.
Melike Aliciaslan +3 more
wiley +1 more source
Hematopoietic (stem) cells—The elixir of life?
FEBS Letters, Volume 600, Issue 4, Page 392-417, February 2026.The aging of HSCs (hematopoietic stem cells) and the blood system leads to the decline of other organs. Rejuvenating aged HSCs improves the function of the blood system, slowing the aging of the heart, kidney, brain, and liver, and the occurrence of age‐related diseases.
Emilie L. Cerezo +4 more
wiley +1 more source
Expression of progerin does not result in an increased mutation rate [PDF]
, 2016 Abstract In the premature ageing disease Hutchinson-Gilford progeria syndrome (HGPS) the underlying genetic defect in the lamin A gene leads to accumulation at the nuclear lamina of progerin – a mutant form of lamin A that cannot be correctly processed.
Deniaud, Emmanuelle +2 more
openaire +1 more source
Assessing the efficacy of protein farnesyltransferase inhibitors in mouse models of progeria
Journal of Lipid Research, 2010 Hutchinson-Gilford progeria syndrome (HGPS) is caused by the accumulation of a farnesylated form of prelamin A (progerin). Previously, we showed that blocking protein farnesylation with a farnesyltransferase inhibitor (FTI) ameliorates the disease ...
Shao H. Yang +5 more
doaj +1 more source
Personalized Models of Biological Barriers and Their Diseases: Recent Progress with Organs‐On‐Chips
Advanced Biology, Volume 10, Issue 2, February 2026.Buck and Bugter et al. explore the architectural diversity and physiological functions of human barrier systems and reveal how organ‐on‐chip platforms, particularly those integrating patient‐derived cells, are advancing barrier disease modeling. They highlight how emerging biological and technological advances can be used to bridge the gap between ...
Franziska Buck +4 more
wiley +1 more source
New therapeutic approaches to HGPS based on progerin inhibition [PDF]
Orphanet Journal of Rare Diseases, 2015 Hutchinson-Gilford Progeria Syndrome (HGPS) is caused by a de novo heterozygous mutation on LMNA gene that leads to accumulation of progerin, a mutant form of prelamin A. HGPS skin fibroblasts are characterized by multiple nuclear defects: nuclear shape abnormalities chromatin structure alterations, increased DNA damage and cell cycle alterations ...
openaire +1 more source