Results 71 to 80 of about 2,732 (185)

Neonatal progeria: increased ratio of progerin to lamin A leads to progeria of the newborn [PDF]

European Journal of Human Genetics, 2012
Hutchinson-Gilford progeria syndrome (HGPS) is an important model disease for premature ageing. Affected children appear healthy at birth, but develop the first symptoms during their first year of life. They die at an average age of 13 years, mostly because of myocardial infarction or stroke.
Janine, Reunert, Rüdiger, Wentzell, Michael, Walter, Sibylle, Jakubiczka, Martin, Zenker, Thomas, Brune, Stephan, Rust, Thorsten, Marquardt   +7 more
openaire   +2 more sources

Are There Common Mechanisms Between the Hutchinson–Gilford Progeria Syndrome and Natural Aging?

Frontiers in Genetics, 2019
The Hutchinson–Gilford progeria syndrome (HGPS) is a premature aging disease caused by mutations of the LMNA gene leading to increased production of a partially processed form of the nuclear fibrillar protein lamin A – progerin.
Vasily V. Ashapkin, Lyudmila I. Kutueva, Svetlana Y. Kurchashova, Igor I. Kireev   +3 more
doaj   +1 more source

Nuclear membrane ruptures underlie the vascular pathology in a mouse model of Hutchinson-Gilford progeria syndrome

JCI Insight, 2021
The mutant nuclear lamin protein (progerin) produced in Hutchinson-Gilford progeria syndrome (HGPS) results in loss of arterial smooth muscle cells (SMCs), but the mechanism has been unclear. We found that progerin induces repetitive nuclear membrane (NM)
Paul H. Kim, Natalie Y. Chen, Patrick J. Heizer, Yiping Tu, Thomas A. Weston, Jared L.-C. Fong, Navjot Kaur Gill, Amy C. Rowat, Stephen G. Young, Loren G. Fong   +9 more
doaj   +1 more source

Cellular Senescence and Aging: Mechanisms, Disease Convergence, and Therapeutic Frontiers

MedComm, Volume 7, Issue 7, July 2026.
This schematic illustrates the hierarchical and interconnected nature of the primary molecular hallmarks of aging. The progression of aging is driven by a convergence of intrinsic molecular insults. Within the nucleus, genomic instability and telomere attrition trigger persistent DDR, accompanied by extensive epigenetic alterations.
Guowei Cai, Jia Ren, Li Wang, Yingying Wu, Huirui Wang, Xiaomeng Cao, Hao Shang, Xuben Hou, Yujiu Wang, Haibo Xue, Ting Dong   +10 more
wiley   +1 more source

Nuclear lamins and progerin are dispensable for antioxidant Nrf2 response to arsenic and cadmium [PDF]

Cellular Signalling, 2017
Lamins are important constituents of the nuclear inner membrane and provide a platform for transcription factors and chromatin. Progerin, a C-terminal truncated lamin A mutant, causes premature aging termed Hutchinson-Gilford Progeria Syndrome (HGPS). Oxidative stress appears to be involved in the pathogenesis of HGPS, although the mechanistic role of ...
Kazunori Hashimoto, Rima Majumdar, Yoshiaki Tsuji   +2 more
openaire   +2 more sources

Temsirolimus increases progerin clearance via autophagy. [PDF]

, 2016
(A) Representative Western blot of control (GMO3349C) and HGPS (HGADFN 003, HGADFN127) fibroblasts that were either mock-treated or Temsirolimus-treated (Tem) for a period of 9 days.
Diana Gabriel (3608093), Karima Djabali (82182), Leslie B. Gordon (82180)   +2 more
core   +1 more source

More Than Skin Deep – the Effects of Ultraviolet Radiation on Cathepsin K and Progerin Expression in Cultured Dermal Fibroblasts

Clinical, Cosmetic and Investigational Dermatology, 2021
Igor Aleksander Bednarski,1 Magdalena Ciążyńska,2 Jacek Kabziński,3 Ireneusz Majsterek,3 Dorota Sobolewska-Sztychny,1 Joanna Narbutt,1 Aleksandra Lesiak1 1Department of Dermatology, Pediatric Dermatology and Dermatological ...
Bednarski IA, Ciążyńska M, Kabziński J, Majsterek I, Sobolewska-Sztychny D, Narbutt J, Lesiak A   +6 more
doaj  

Autophagic degradation of farnesylated prelamin A as a therapeutic approach to lamin-linked progeria

European Journal of Histochemistry, 2011
Farnesylated prelamin A is a processing intermediate produced in the lamin A maturation pathway. Accumulation of a truncated farnesylated prelamin A form, called progerin, is a hallmark of the severe premature ageing syndrome, Hutchinson-Gilford progeria.
V. Cenni, C. Capanni, M. Columbaro, M. Ortolani, M.R. D'Apice, G. Novelli, M. Fini, S. Marmiroli, E. Scarano, N.M. Maraldi, S. Squarzoni, S. Prencipe, G. Lattanzi   +12 more
doaj   +1 more source

Farnesyltransferase Deficiency in Cardiomyocytes Initiates Senescence and Contributes to Cardiac Fibrosis

Advanced Science, Volume 13, Issue 26, 8 May 2026.
Lipid overload suppresses SREBF2‐mediated FNTB expression, leading to defective Lamin A maturation and nuclear envelope instability. This nuclear catastrophe triggers a pro‐fibrotic senescence program in cardiomyocytes. Notably, restoring nuclear integrity via AAV9‐based gene therapy effectively attenuates cardiac remodeling, identifying the ...
Yuxiao Chen, Lian Lou, Xuan Zhang, Lin Yan, Qi Zhang, Wen Shi, Jie Ding, Xu Lin, Rong Jiang, Shuo Liu, Thida Sok, Pengli Wang, Yun Mou, Jie Han, Shenjiang Hu, Xiaogang Guo, Jian Yang   +16 more
wiley   +1 more source

A new connection between VHL and cancer threads through progerin [PDF]

Cell Cycle, 2013
A-type nuclear lamins, all encoded by the LMNA gene through alternative splicing, are targets for mutation in a wide range of rare diseases, including a dominant mutation that enhances production of lamin A splice variant progerin, causing Hutchinson-Gilford progeria syndrome (HGPS).1 Often ignored, however, is the complex and poorly understood ...
openaire   +2 more sources