Results 11 to 20 of about 4,214 (205)

MG132 Induces Progerin Clearance and Improves Disease Phenotypes in HGPS-Like Patients’ Cells [PDF]

Cells, 2022
Progeroid syndromes (PS), including Hutchinson-Gilford Progeria Syndrome (HGPS), are premature and accelerated aging diseases, characterized by clinical features mimicking physiological aging.
Nicolas Levy, Koffi M Guedenon
exaly   +4 more sources

Progerin, an Aberrant Spliced Form of Lamin A, Is a Potential Therapeutic Target for HGPS

Cells, 2023
Hutchinson–Gilford progeria syndrome (HGPS) is an extremely rare genetic disorder caused by the mutant protein progerin, which is expressed by the abnormal splicing of the LMNA gene.
Bum-Joon Park, So-Mi Kang, Tae-Gyun Woo
exaly   +4 more sources

Hutchinson-Gilford progeria syndrome alters the endothelial genetic response to laminar shear stress [PDF]

Frontiers in Physiology
IntroductionHutchinson-Gilford Progeria Syndrome (HGPS) is a fatal, accelerated-aging disease caused by a mutation in the nuclear envelope protein Lamin A.
Crystal C. Kennedy, Jonathan L. Carter, George A. Truskey, George A. Truskey   +3 more
doaj   +2 more sources

Mesenchymal stem cells derived from patients with premature aging syndromes display hallmarks of physiological aging

Life Science Alliance, 2022
Using of genome-wide approaches on models from laminopathies with or without progerin accumulation (HGPS, HGPS-L, APS), this study provides new insights on pathways altered during early stages of mesenchymal stem cells differentiation.
Jean Philippe Trani, Raphaël Chevalier, Leslie Caron, Claire El Yazidi, Natacha Broucqsault, Léa Toury, Morgane Thomas, Karima Annab, Bernard Binetruy, Annachiara De Sandre-Giovannoli, Nicolas Levy, Frédérique Magdinier, Jérôme D Robin   +12 more
doaj   +2 more sources

Molecular and Cellular Mechanisms Driving Cardiovascular Disease in Hutchinson-Gilford Progeria Syndrome: Lessons Learned from Animal Models [PDF]

Cells, 2021
Hutchinson-Gilford progeria syndrome (HGPS) is a rare genetic disease that recapitulates many symptoms of physiological aging and precipitates death. Patients develop severe vascular alterations, mainly massive vascular smooth muscle cell loss, vessel ...
Ignacio Benedicto, Beatriz Dorado, Vicente Andrés   +2 more
doaj   +2 more sources

Extracellular matrix abnormalities in Hutchinson–Gilford progeria fibroblasts: a specific defect in collagen IV and basement membrane architecture? [PDF]

Matrix Biology Plus
Hutchinson–Gilford progeria (HGPS) is a rare genetic disorder characterized by clinical features that mimic accelerated aging. The classical form of progeria is caused by a heterozygous pathogenic variant in in the LMNA gene resulting in the truncated ...
Shreya Karmacharya, Arthur Lauri Pasanen-Zentz, Franziska Busse, Nils Michael Kronenberg, Diego Rodrigo Alvarez Chavez, Suzan Al-Gburi, Tristan Lerbs, Bent Brachvogel, Clara Velmans, Iliana Tantcheva-Poor, Raimund Wagener, Mats Paulsson, Carien M. Niessen, Malte Christian Gather, Thomas Krieg, Alvise Schiavinato   +15 more
doaj   +2 more sources

Single-cell RNA sequencing analysis reveals the critical role of fibroblasts in aortic progeria-associated vascular remodeling in Hutchinson-Gilford progeria syndrome mice [PDF]

Frontiers in Immunology
BackgroundPatients with Hutchinson-Gilford progeria syndrome (HGPS) typically succumb to cardiovascular diseases in their teens. Although fibroblasts have been implicated in the progression of arteriosclerosis, their roles and mechanisms in progeroid ...
Qian Sun, Qian Sun, Liehao Yang, Liehao Yang, Zilong Zhou, Nan Wu, Chunyi Li, Qianying Hu, Xianling Cong, Xianling Cong   +9 more
doaj   +2 more sources

Hemoglobinopathies and iron deficiency among Northeast-Thai blood donors deferred for low hemoglobin [PDF]

Scientific Reports
While iron deficiency (ID) is well-known as the main reason for low hemoglobin (Hb) deferral among blood donors, the contribution of hemoglobinopathies (HgPs) has been overlooked.
Tassaneewan Chueajetton, Hataichanok Srivorakun, Pattara Sanchaisuriya, Kanokwan Sanchaisuriya   +3 more
doaj   +2 more sources

General anesthesia in patient with Hutchinson-Gilford Progeria syndrome: two case reports of dental treatment in the one patient [PDF]

BMC Anesthesiology
Background Hutchinson-Gilford progeria syndrome (HGPS) is a rare genetic disorder that manifests itself in premature aging. Craniofacial and skeletal abnormalities, cardiovascular pathology, concomitant cerebrovascular diseases, and potential airway ...
B. Mankovsky, O. Halushko, Y. Zablotskyy, M. Synytsyn   +3 more
doaj   +2 more sources

Vulnerability of progeroid smooth muscle cells to biomechanical forces is mediated by MMP13

Nature Communications, 2020
Hutchinson-Gilford Progeria Syndrome (HGPS) is a premature aging disease and smooth muscle cells are the most affected cells in HGPS individuals. Here, the authors report a microfluidics platform with HGPS induced pluripotent stem cells and show that ...
Patricia R. Pitrez, Luís Estronca, Luís Miguel Monteiro, Guillem Colell, Helena Vazão, Deolinda Santinha, Karim Harhouri, Daniel Thornton, Claire Navarro, Anne-Laure Egesipe, Tânia Carvalho, Rodrigo L. Dos Santos, Nicolas Lévy, James C. Smith, João Pedro de Magalhães, Alessandro Ori, Andreia Bernardo, Annachiara De Sandre-Giovannoli, Xavier Nissan, Anna Rosell, Lino Ferreira   +20 more
doaj   +2 more sources