Results 61 to 70 of about 2,732 (185)
Impact of MnTBAP and Baricitinib Treatment on Hutchinson–Gilford Progeria Fibroblasts
Pharmaceuticals, 2022 Hutchinson–Gilford progeria syndrome (HGPS) is a rare premature aging disease. It is caused by a mutation in the LMNA gene, which results in a 50-amino-acid truncation of prelamin A.
Elena Vehns +2 more
doaj +1 more source
Cardiovascular Progerin Suppression and lamin A Restoration Rescues Hutchinson-Gilford Progeria Syndrome. [PDF]
, 2021 Background: Hutchinson-Gilford progeria syndrome (HGPS) is a rare disorder characterized by premature aging and death mainly due to myocardial infarction, stroke, or heart failure.
Anna Barkaway +53 more
core +1 more source
Oncology Reports, 2017 Ultraviolet (UV) radiation is the primary risk factor underlying photoaging and photocarcinogenesis. Mounting research has focused on the role of DNA damage response pathways in UV-induced double-strand break (DSB) repair. In the present study, we hypothesized that UVA-induced aberrant progerin upregulation may adversely affect p53-binding protein 1 ...
Xin, Huang +6 more
openaire +3 more sources
DEVELOPMENT OF AN ACCELERATED ALZHEIMER’S DISEASE IN VITRO MODEL WITH THE ADDITION OF PROGERIN [PDF]
, 2023 Alzheimer’s Disease (AD) is one of the most common causes of dementia. Twopathological features of AD include amyloid plaques and neurofibrillary tangles. The mechanism underlying the disease's onset and progression remains unclear.
Xue, Huijing
core +1 more source
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
Aging Cell, Volume 25, Issue 8, August 2026.We demonstrated that progerin induces the formation of autophagosomes but impairs their maturation and subsequent fusion with lysosomes. Progerin impedes proper autophagy flux, thus preventing its own autophagic degradation. Selinexor treatment improved both autophagosome maturation and autophagosome‐lysosome fusion, which ultimately led to effective ...
Ian García‐Aguirre +18 more
wiley +1 more source
Progerin accumulation in human elderly skin biopsy sections. [PDF]
, 2013 A, Forehead skin section from a 69-year-old individual probed with anti-progerin antibody and counterstained with dapi. Bars correspond to 100 and 50 µm, respectively. B, Forehead skin section from a 93-year-old donor.
Desiree Ratner (82177) +6 more
core +1 more source
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
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