Results 71 to 80 of about 9,936 (203)
The Application of a Statistical Model Investigating Reactive Oxygen Species in Premature Ageing Syndromes [PDF]
The premature ageing syndromes Hutchison-Gilford Progeria Syndrome (HGPS) and Restrictive Dermopathy (RD) are rare genetic disorders that result in greatly accelerated ageing. RD is neonatal fatal, whereas children suffering from HGPS age approximately 8
MUTER, JOANNE,RUTH
core
Cellular Senescence and Aging: Mechanisms, Disease Convergence, and Therapeutic Frontiers
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 +10 more
wiley +1 more source
Single Cell Mechanics in Disease Progression
Cells transmit distinct mechanical forces through ECM adhesion and cell–cell junctions and actomyosin‐generated traction forces are transmitted to the substrate through integrin‐based focal adhesions. Mechanical signals are further transmitted to the nucleus via the LINC complex, connecting the cytoskeleton to the nuclear lamina. These forces integrate
Sabin Kim +3 more
wiley +1 more source
Identification of mitochondrial dysfunction in Hutchinson-Gilford progeria syndrome through use of stable isotope labeling with amino acids in cell culture [PDF]
Work in the author's laboratories is supported by grants from the Spanish Ministry of Economy and Competiveness(MINECO)(SAF2010-16044; SAF2011-23089, SAF2009-08007, CSD2007-00020, CTQ2010-18644), Instituto de Salud Carlos III(ISCIII) (RD06/0014/0021 ...
Rivera Torres, José +17 more
core +1 more source
Background/Objectives: Hutchinson–Gilford progeria syndrome (HGPS) is a rare genetic disorder that cause premature aging due to LMNA mutations and progerin accumulation.
Eun-Young Joo +6 more
doaj +1 more source
Generation and characterization of a novel knockin minipig model of Hutchinson-Gilford progeria syndrome [PDF]
Hutchinson-Gilford progeria syndrome (HGPS) is an extremely rare genetic disorder for which no cure exists. The disease is characterized by premature aging and inevitable death in adolescence due to cardiovascular complications.
Gonzalo, Pilar +20 more
core +1 more source
Posttranslational Modifications of Lamin A/C and Cardiac Aging: A Short Review
Cardiac aging is considered as natural and age-related changes in the heart, which can lead to a decline in heart function. Cardiac aging increases the risk of heart failure, atrial fibrillation, and other cardiovascular complications.
Vikas Tiwari +2 more
doaj +1 more source
Management of cataract in Werner syndrome
Werner syndrome (WS) is a rare progressive disorder. It is characterized by the appearance of unusually accelerated aging (progeria) including bilateral senile cataract. Here, we report a successful management of hypermature cataract in WS.
Rakhi Kusumesh +3 more
doaj +1 more source
New drug treatments in progeria cells: modulating mutated lamins to improve cell quality
Thirteen year study on lamin A and prelamin A biology have shown that the lamin A to prelamin A ratio is important to determine both physiological and pathological pathways.
Stefano Squarzoni, Giovanna Lattanzi
core
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 +9 more
doaj +1 more source

