Results 21 to 30 of about 7,722 (240)

iPSC-Derived Endothelial Cells Affect Vascular Function in a Tissue-Engineered Blood Vessel Model of Hutchinson-Gilford Progeria Syndrome

Stem Cell Reports, 2020
Summary: Hutchinson-Gilford progeria syndrome (HGPS) is a rare disorder caused by a point mutation in the Lamin A gene that produces the protein progerin. Progerin toxicity leads to accelerated aging and death from cardiovascular disease.
Leigh Atchison, Nadia O. Abutaleb, Elizabeth Snyder-Mounts, Yantenew Gete, Alim Ladha, Thomas Ribar, Kan Cao, George A. Truskey   +7 more
doaj   +1 more source

miR-376a-3p and miR-376b-3p overexpression in Hutchinson-Gilford progeria fibroblasts inhibits cell proliferation and induces premature senescence

iScience, 2022
Summary: Hutchinson-Gilford progeria syndrome (HGPS) is a rare genetic disorder, in which an abnormal and toxic protein called progerin, accumulates in cell nuclei, leading to major cellular defects.
Diane Frankel, Valérie Delecourt, Elva-María Novoa-del-Toro, Jérôme D. Robin, Coraline Airault, Catherine Bartoli, Aurélie Carabalona, Sophie Perrin, Kilian Mazaleyrat, Annachiara De Sandre-Giovannoli, Frederique Magdinier, Anaïs Baudot, Nicolas Lévy, Elise Kaspi, Patrice Roll   +14 more
doaj   +1 more source

Vascular Smooth Muscle Cell-Specific Progerin Expression Provokes Contractile Impairment in a Mouse Model of Hutchinson-Gilford Progeria Syndrome that Is Ameliorated by Nitrite Treatment

Cells, 2020
Cardiovascular disease (CVD) is the main cause of death worldwide, and aging is its leading risk factor. Aging is much accelerated in Hutchinson−Gilford progeria syndrome (HGPS), an ultra-rare genetic disorder provoked by the ubiquitous expression ...
Lara del Campo, Amanda Sánchez-López, Cristina González-Gómez, María Jesús Andrés-Manzano, Beatriz Dorado, Vicente Andrés   +5 more
doaj   +1 more source

Impact of Progerin Expression on Adipogenesis in Hutchinson—Gilford Progeria Skin-Derived Precursor Cells

Cells, 2021
Hutchinson–Gilford progeria syndrome (HGPS) is a segmental premature aging disease caused by a mutation in LMNA. The mutation generates a truncated and farnesylated form of prelamin A, called progerin.
Farah Najdi, Peter Krüger, Karima Djabali   +2 more
doaj   +1 more source

A small-molecule ICMT inhibitor delays senescence of Hutchinson-Gilford progeria syndrome cells

eLife, 2021
A farnesylated and methylated form of prelamin A called progerin causes Hutchinson-Gilford progeria syndrome (HGPS). Inhibiting progerin methylation by inactivating the isoprenylcysteine carboxylmethyltransferase (ICMT) gene stimulates proliferation of ...
Xue Chen, Haidong Yao, Muhammad Kashif, Gwladys Revêchon, Maria Eriksson, Jianjiang Hu, Ting Wang, Yiran Liu, Elin Tüksammel, Staffan Strömblad, Ian M Ahearn, Mark R Philips, Clotilde Wiel, Mohamed X Ibrahim, Martin O Bergo   +14 more
doaj   +1 more source

Effect of Magnesium on Replication of Rhinovirus HGP [PDF]

Journal of Virology, 1967
It is known that plaque formation by some rhinoviruses is greatly enhanced by increasing the concentration of MgCl 2 . The mechanism of this action was studied by investigating the effects of MgCl 2 on rhinovirus HGP adsorption, growth, clumping, thermal stability, and cell susceptibility to ...
M, Fiala, G E, Kenny
openaire   +2 more sources

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, Rouven Arnold, Karima Djabali   +2 more
doaj   +1 more source

Altered behavior with an organic cause: a case report

Journal of Medical Case Reports, 2019
Background Insulinomas are pancreatic endocrine tumors of rare incidence worldwide, the vast majority are of single occurrence and benign. These may not always present with the clear symptoms described in the literature and may be overlooked because ...
Dolores Mejía, Rafael Contreras, Guarina Molina, Michael Alcántara   +3 more
doaj   +1 more source

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   +1 more source

Metabolic Dysfunction in Hutchinson–Gilford Progeria Syndrome

Cells, 2020
Hutchinson−Gilford Progeria Syndrome (HGPS) is a segmental premature aging disease causing patient death by early teenage years from cardiovascular dysfunction.
Ray Kreienkamp, Susana Gonzalo
doaj   +1 more source