Results 61 to 70 of about 2,950 (183)
Aging Cell, Volume 25, Issue 8, August 2026.Skeletal muscle aging is associated with a decrease in expression of the lactate receptor GPR81. Treating senescent myoblasts in vitro and progeric mice in vivo with GPR81 agonist decreases intramyocellular lipid accumulation and reverses hallmarks of aging. ABSTRACT Skeletal muscle aging is associated with increased lipid accumulation, or myosteatosis,
Pihu Mehrotra +11 more
wiley +1 more source
Progerin expression in humans: Implications for natural ageing
Mechanisms of Ageing and DevelopmentProgerin, 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 +4 more
openaire +2 more sources
Nuclear import pathway key to rescuing dominant progerin phenotypes [PDF]
Science Signaling, 2018 Restoring a nuclear import pathway rescues pathological phenotypes of cells from HGPS patients (Larrieuet al., in 3 July 2018 issue).
openaire +2 more sources
Scientific Reports, 2021 Children with Hutchinson–Gilford Progeria Syndrome (HGPS) suffer from multiple cardiovascular pathologies due to the expression of progerin, a mutant form of the nuclear envelope protein Lamin A.
Ryan von Kleeck +5 more
doaj +1 more source
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 +3 more
doaj +1 more source
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 +9 more
doaj +1 more source
Targeting RANKL Prevents Bone Loss, Improves Muscle Function and Extends Lifespan in Progeroid Mice
Aging Cell, Volume 25, Issue 8, August 2026.Targeting of RANKL by genetic and pharmacological approaches ameliorates key features of the progeroid phenotype in Zmpste24−/− mice. RANKL intervention restores bone mass, improves muscle phenotype, and extends survival. These findings support further exploration of RANKL‐targeted therapies for Hutchinson‐Gilford progeria syndrome.
Sandra Freitas‐Rodríguez +11 more
wiley +1 more source
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 +6 more
doaj
Advanced Functional Materials, Volume 36, Issue 59, 23 July 2026.This article details the development of an artery‐on‐chip platform for in vitro arterial disease modeling and therapeutic discovery. It describes the fabrication of a fibrin biomaterial scaffold seeded with iPSC‐derived smooth muscle and endothelial cells, mimicking native artery properties. Two genetic disease models showcase the platform's ability to
Danielle Yarbrough +10 more
wiley +1 more source
Nuclear dysfunction in aging and neurodegeneration
Alzheimer's &Dementia, Volume 22, Issue 7, July 2026.Abstract Neurodegenerative diseases are characterized by a loss of neuronal function and structure, often in a region‐specific manner. Multiple factors contribute to neuronal dysfunction and death, including pathogenic protein buildup, protein mislocalization, and inflammation. Despite extensive research, the common mechanisms driving neurodegeneration
Abbigael Aday +7 more
wiley +1 more source