Results 71 to 80 of about 5,892 (251)
Vascular Smooth Muscle-Specific Progerin Expression Accelerates Atherosclerosis and Death in a Mouse Model of Hutchinson-Gilford Progeria Syndrome [PDF]
, 2018 Background: Progerin, an aberrant protein that accumulates with age, causes the rare genetic disease Hutchinson-Gilford progeria syndrome (HGPS). Patients who have HGPS exhibit ubiquitous progerin expression, accelerated aging and atherosclerosis, and ...
Andrés Manzano, María J. +7 more
core +4 more sources
Hutchinson-Gilford Progeria Syndrome (HGPS)
Journal of Oral Diagnosis, 2016 Hutchinson-Gilford Progeria Syndrome (HGPS) is an extremely rare genetic condition characterized by premature aging and it’s about one case for every four to eight million people. Children affected usually have premature death due to cardiovascular problems.
Alexandre Simoes Nogueira +6 more
openaire +1 more source
The Galactic sky through H.E.S.S. eyes
, 2015 The High Energy Stereoscopic System (H.E.S.S.) is an array of five imaging atmospheric Cherenkov telescopes. Since 2003 it has been operating in the configuration of four 12 m telescopes complemented in 2012 by a much bigger 28 m telescope in the centre ...
Sushch, I.
core +1 more source
Mouse models for understanding the molecular mechanism of bone disease in Hutchinson-Gilford progeria syndrome [PDF]
, 2011 Aging is a complex process affecting all people. Intense research is applied to elucidate the biological basis of aging and disease that develop with aging.
Schmidt, Eva
core +1 more source
Biology Open, 2012 Summary Hutchinson-Gilford progeria syndrome (HGPS, OMIM 176670) is a rare disorder characterized by segmental accelerated aging and early death from coronary artery disease or stroke. Nearly 90% of HGPS sufferers carry a G608G mutation within exon 11 of
Vera Wenzel +7 more
doaj +1 more source
Vascular disease modeling using induced pluripotent stem cells: Focus in Hutchinson-Gilford Progeria Syndrome [PDF]
, 2016 Transparency document related to this article can be found online at http://dx.doi.org/10.1016/j.bbrc.2015.10.014Induced pluripotent stem cells (iPSCs) represent today an invaluable tool to create disease cell models for modeling and drug screening ...
Ferreira, L. +3 more
core +1 more source
First Generation Proteolysis Targeting Chimeras (PROTACs) for the Treatment of Progeria
Advanced Science, EarlyView.We report the first PROTACs designed to degrade progerin, introducing a novel therapeutic approach for progeria. The best compound, UCM‐18142, significantly reduces progerin levels and improves key disease phenotypes in patient‐derived cells and in the LmnaG609G/G609G mouse model, paving the way for new treatment strategies targeting the root cause of ...
Jon Macicior‐Michelena +5 more
wiley +1 more source
Epigenetic deregulation of lamina-associated domains in Hutchinson-Gilford progeria syndrome
Genome Medicine, 2020 Background Hutchinson-Gilford progeria syndrome (HGPS) is a progeroid disease characterized by the early onset of age-related phenotypes including arthritis, loss of body fat and hair, and atherosclerosis. Cells from affected individuals express a mutant
Florian Köhler +9 more
doaj +1 more source
IEEE Access, 2023 Multi-vector smart local energy systems are playing an increasingly importantly role in the fast-track decarbonisation of our global energy services. An emergent contributor to global decarbonisation is green hydrogen. Green hydrogen can remove or reduce
Hani Gharavi Ahangar +2 more
doaj +1 more source
Understanding Health and Disease with Multidimensional Single-Cell Methods
, 2013 Current efforts in the biomedical sciences and related interdisciplinary fields are focused on gaining a molecular understanding of health and disease, which is a problem of daunting complexity that spans many orders of magnitude in characteristic length
Banavar, Jayanth R. +2 more
core +1 more source