Results 101 to 110 of about 2,732 (185)

A Cell-Intrinsic Interferon-like Response Links Replication Stress to Cellular Aging Caused by Progerin

Cell Reports, 2018
Summary: Hutchinson-Gilford progeria syndrome (HGPS) is a premature aging disease caused by a truncated lamin A protein (progerin) that drives cellular and organismal decline.
Ray Kreienkamp, Simona Graziano, Nuria Coll-Bonfill, Gonzalo Bedia-Diaz, Emily Cybulla, Alessandro Vindigni, Dale Dorsett, Nard Kubben, Luis Francisco Zirnberger Batista, Susana Gonzalo   +9 more
doaj   +1 more source

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

PCR strategy for the detection of progerin expression in heart and blood. [PDF]

, 2018
A Schematic overview of PCR based strategy to analyze progerin mRNA expression in human heart and the blood samples. Shown are the consensus donor splice sequence at the end of exon 11, the sequence of the normal LMNA cryptic splice site, and two common ...
Santhosh Kumar Ghadge (5150576), Andreas Wimmer (2813278), Valentina Goetsch (5150567), Moritz Messner (5150570), Jakob Dörler (5150573), Gerhard Pölzl (5150579), Marc-Michael Zaruba (5150564)   +6 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

Progerin expression in humans: Implications for natural ageing

Mechanisms of Ageing and Development
Progerin, a truncated lamin A isoform generated by cryptic LMNA splicing, is the pathogenic driver of Hutchinson-Gilford Progeria Syndrome (HGPS) and has been implicated as a putative marker in natural ageing. Low-level progerin arises in normal tissues, particularly skin, vasculature, and blood-derived cells, where it contributes to nuclear ...
So-mi Kang, Soyoung Park, Tae-Gyun Woo, Bae-Hoon Kim, Bum-Joon Park   +4 more
openaire   +2 more sources

Temsirolimus Partially Rescues the Hutchinson-Gilford Progeria Cellular Phenotype.

PLoS ONE, 2016
Hutchinson-Gilford syndrome (HGPS, OMIM 176670, a rare premature aging disorder that leads to death at an average age of 14.7 years due to myocardial infarction or stroke, is caused by mutations in the LMNA gene.
Diana Gabriel, Leslie B Gordon, Karima Djabali   +2 more
doaj   +1 more source

Organ‐Specific Dedifferentiation and Epigenetic Remodeling in In Vivo Reprogramming

Aging Cell, Volume 24, Issue 11, November 2025.
Transient in vivo expression of Yamanaka factors (OSKM) enhances regenerative competence and rejuvenation across multiple tissues. We highlight mechanistic links between injury‐induced dedifferentiation and OSKM reprogramming, while emphasizing safety challenges and the need for precise spatiotemporal control to enable clinical translation.
Beom‐Ki Jo, Seung‐Yeon Lee, Hee‐Ji Eom, Jumee Kim, Hyuk‐Jin Cha   +4 more
wiley   +1 more source

Progerin phosphorylation in interphase is lower and less mechanosensitive than lamin-A,C in iPS-derived mesenchymal stem cells [PDF]

, 2018
Interphase phosphorylation of lamin-A,C depends dynamically on a cell's microenvironment, including the stiffness of extracellular matrix. However, phosphorylation dynamics is poorly understood for diseased forms such as progerin, a permanently ...
Yuntao Xia (5577878), Sangkyun Cho (5577869), Dennis E. Discher (436172), Irena L. Ivanovska (5577875), Amal Abbas (5577872), Jerome Irianto (4902721), Manu Tewari (5577881)   +6 more
core   +1 more source

Impairment of nuclear F-actin formation and its relevance to cellular phenotypes in Hutchinson-Gilford progeria syndrome

Nucleus, 2020
Hutchinson-Gilford progeria syndrome (HGPS) is a premature aging disorder caused by a mutation of lamin A, which contributes to nuclear architecture and the spatial organization of chromatin in the nucleus.
Yuto Takahashi, Shogo Hiratsuka, Nanako Machida, Daisuke Takahashi, Junpei Matsushita, Pavel Hozak, Tom Misteli, Kei Miyamoto, Masahiko Harata   +8 more
doaj   +1 more source

Autophagic Removal of Farnesylated Carboxy-Terminal Lamin Peptides

Cells, 2018
The mammalian nuclear lamina proteins—prelamin A- and B-type lamins—are post-translationally modified by farnesylation, endoproteolysis, and carboxymethylation at a carboxy-terminal CAAX (C, cysteine; a, aliphatic amino acid; X, any amino ...
Xiang Lu, Karima Djabali
doaj   +1 more source