Results 91 to 100 of about 3,816 (187)

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, Sandy Y. Chang, Douglas A. Andres, H. Peter Spielmann, Stephen G. Young, Loren G. Fong   +5 more
doaj   +1 more source

Ignoring the planet: A critical blind spot for research on ageing

Journal of Internal Medicine, Volume 298, Issue 6, Page 578-590, December 2025.
Abstract Although research on ageing has largely concentrated on understanding the fundamental biology of the ageing process and devising pharmaceutical interventions in order to slow it down, increasing evidence has underscored the crucial role of environmental inputs across the life course and across generations, in shaping both individual and ...
Paul Shiels, Ognian Neytchev, Gillian Borland, Polina Germushkina, Richard Johnson, Peter Stenvinkel, Tina Woods   +6 more
wiley   +1 more source

Progerin-Induced Replication Stress Facilitates Premature Senescence in Hutchinson-Gilford Progeria Syndrome [PDF]

Molecular and Cellular Biology, 2017
Hutchinson-Gilford progeria syndrome (HGPS) is caused by a mutation in LMNA that produces an aberrant lamin A protein, progerin. The accumulation of progerin in HGPS cells leads to an aberrant nuclear morphology, genetic instability, and p53-dependent premature senescence. How p53 is activated in response to progerin production is unknown. Here we show
Keith, Wheaton, Denise, Campuzano, Weili, Ma, Michal, Sheinis, Brandon, Ho, Grant W, Brown, Samuel, Benchimol   +6 more
openaire   +2 more sources

Rejuvenation by cell reprogramming: A new horizon in gerontology [PDF]

, 2018
The discovery of animal cloning and subsequent development of cell reprogramming technology were quantum leaps as they led to the achievement of rejuvenation by cell reprogramming and the emerging view that aging is a reversible epigenetic process. Here,
Brown, Oscar Alfredo, Canatelli Mallat, Martina, Chiavellini, Priscila, Goya, Rodolfo Gustavo, Hereñú, Claudia Beatriz, Lehmann, Marianne   +5 more
core   +2 more sources

Decoding Dental Stem Cell Aging: Mechanisms, Therapeutic Strategies, and Beyond

Advanced Science, Volume 12, Issue 44, November 27, 2025.
Dental stem cell (DSC) aging involves genomic instability, mitochondrial dysfunction, telomere attrition, and epigenetic alterations, leading to impaired proliferation, reduced differentiation potential, and pro‐inflammatory secretory activity. These processes drive cellular senescence and compromise regenerative and immunomodulatory functions, thereby
Xinyuan Zhao, Yunfan Lin, Pei Lin, Ye Lu, Jiarong Zheng, Xu Chen, Zihao Zhou, Li Cui   +7 more
wiley   +1 more source

The Consideration of Pseudoxanthoma Elasticum as a Progeria Syndrome

Frontiers in Bioscience-Landmark, 2023
Background: Pseudoxanthoma elasticum (PXE) is a rare autosomal recessive disorder caused by mutations in the ATP-binding cassette sub-family C member 6 (ABCC6) gene.
Janina Tiemann, Christopher Lindenkamp, Thomas Wagner, Andreas Brodehl, Ricarda Plümers, Isabel Faust-Hinse, Cornelius Knabbe, Doris Hendig   +7 more
doaj   +1 more source

The truncated prelamin A in Hutchinson-Gilford progeria syndrome alters segregation of A-type and B-type lamin homopolymers. [PDF]

, 2006
Hutchinson-Gilford progeria syndrome (HGPS) is a dominant autosomal premature aging syndrome caused by the expression of a truncated prelamin A designated progerin.
Buendia, Brigitte, Coppey-Moisan, Maïté, Courvalin, Jean-Claude, Delbarre, Erwan, Gaillard, Claire, Tramier, Marc   +5 more
core   +1 more source

New insights into applications of base editor in hereditary disorders

Interdisciplinary Medicine, Volume 3, Issue 6, November 2025.
Abstract Hereditary disorders are a group of diseases caused by genetic mutations or chromosomal variations. Although the incidence of each genetic disorder is relatively low, patients affected by the disease generally experience a range of severe symptoms, including blindness, disability, and even premature death. In addition, the available treatments
Maoping Cai, Linhui Zhang, Liqing Li, Yue Liu, Canbin Lv, Yan Shi, Jianyuan Zhao, Dingwei Ye, Yuanyuan Qu   +8 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

Human iPSC-Based Modeling of Late-Onset Disease via Progerin-Induced Aging [PDF]

Cell Stem Cell, 2013
Reprogramming somatic cells to induced pluripotent stem cells (iPSCs) resets their identity back to an embryonic age and, thus, presents a significant hurdle for modeling late-onset disorders. In this study, we describe a strategy for inducing aging-related features in human iPSC-derived lineages and apply it to the modeling of Parkinson's disease (PD).
Miller, Justine D., Ganat, Yosif M., Kishinevsky, Sarah, Bowman, Robert L., Liu, Becky, Tu, Edmund Y., Mandal, Pankaj K., Vera, Elsa, Shim, Jae-won, Kriks, Sonja, Taldone, Tony, Fusaki, Noemi, Tomishima, Mark J., Krainc, Dimitri, Milner, Teresa A., Rossi, Derrick J., Studer, Lorenz   +16 more
openaire   +2 more sources