Results 71 to 80 of about 7,722 (240)

Wiederherstellen der Proteinabbauwege im Hutchinson-Gilford Progerie Syndrom (HGPS) [PDF]

, 2019
The Hutchinson-Gilford progeria syndrome (HGPS) is a rare premature aging disorder that leads to death at an average age of 14.7 years. A point mutation within the lamin A gene results in the accumulation of a mutant protein called progerin.
Gabriel, Diana
core  

Temsirolimus ameliorates lamin status and nuclear shape in HGPS cells. [PDF]

, 2016
(A) Representative Western blots of lamin A/C, progerin, and β-actin in control and HGPS total cell extracts isolated from either mock-treated cells or cells treated with 1.0 μM Temsirolimus daily for a period of 9 days.
Diana Gabriel (3608093), Karima Djabali (82182), Leslie B. Gordon (82180)   +2 more
core   +1 more source

Chd4 and ThPOK cooperate to preserve structural and electrophysiological integrity of the adult heart through Sprr1a repression

The FEBS Journal, EarlyView.
Chd4/NuRD and ThPOK cooperate to maintain transcriptional repression and nuclear organization in adult cardiomyocytes. Chd4 loss reduces miR‐150‐5p, relieving repression of Sprr1a, while ThPOK loss further enhances Sprr1a activation, possibly through altered chromatin–lamina interactions.
Fadoua El Abdellaoui‐Soussi, Apolonia Novillo, Rocío Brea, Marta Ysbert, Lorena Cussó, Gonzalo Pizarro‐Sánchez, Manuel Desco, Iván Hernández, Ángel Luis Armesilla, Ignacio Pérez de Castro, Alicia R. Folgueras, Juan Miguel Redondo, Pablo Gómez‐del Arco   +12 more
wiley   +1 more source

Hutchinson-Gilford Progeria Syndrome: Cellular Mechanisms and Therapeutic Perspectives.

Archives of Medical Research, 2023
In humans, aging is characterized by a gradual decline of physical and psychological functions, with the concomitant onset of chronic-degenerative diseases, which ultimately lead to death.
B. Cisneros, Ian A García-Aguirre, M. De Ita, I. Arrieta-Cruz, H. Rosas-Vargas   +4 more
semanticscholar   +1 more source

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, Carlos Bruno Pinheiro Nogueira, Rômulo Medeiros, Ana Cristina Beviláqua, Paulo Sérgio da Silva Santos, Izabel Regina Fischer Rubira-Bullen, Eduardo Sanches Gonçales   +6 more
openaire   +1 more source

Estrogen Receptor Beta Activation Coordinates Liver Lipid Remodeling and Metabolic Fluxes, Preventing Lipotoxicity

Comprehensive Physiology, Volume 16, Issue 4, August 2026.
ERβ activation restores glucose and lipid homeostasis by remodeling the hepatic lipidome. Mechanistically, it promotes complete fatty acid oxidation and decreases Acetyl‐CoA levels, leading to reduced ketogenesis and lipotoxicity, as demonstrated through in vivo and in vitro approaches.
Debora Santos Rocha, Eloisa Vilas‐Boas, Camille C. Caldeira da Silva, Everton L. Vogt, Marcos Yoshinaga, Mariana Pacheco Teixeira de Carvalho, Maiara I. C. Queiroz, Tiago Eugênio Oliveira da Silva, Sayuri Miyamoto, Alicia J. Kowaltowski   +9 more
wiley   +1 more source

Redistribution of Sediment and Particulate Mercury Within Upstream Reaches of Galveston Bay in Response to Hurricane Harvey

Journal of Geophysical Research: Oceans, Volume 131, Issue 8, August 2026.
Abstract Extreme storms and flooding can remobilize legacy contaminants stored in the sediment bed; however, event‐scale models that resolve the transport of these materials remain underdeveloped. This study developed a numerical model of contaminated sediment transport during Hurricane Harvey (August 2017), which struck the upstream reaches of ...
Zhiyun Du, Courtney K. Harris, Roberta Southwood, Dongxiao Yin, Daoyang Bao, Fei Ye, Timothy M. Dellapenna, Kehui Xu, Z. George Xue   +8 more
wiley   +1 more source

Repression of the antioxidant NRF2 pathway in premature aging

Cell, 2016
Hutchinson-Gilford Progeria Syndrome (HGPS) is a rare, invariably fatal premature aging disorder. The disease is caused by constitutive production of progerin, a mutant form of the nuclear architectural protein lamin A, leading, through unknown ...
Nard Kubben, Weiqi Zhang, Lixia Wang, T. Voss, Jiping Yang, J. Qu, Guang-Hui Liu, T. Misteli   +7 more
semanticscholar   +1 more source

The amplification of gammaH2AX signal is reduced in G0/G1 phase HGPS cells after Dox and CPT treatment. [PDF]

, 2016
(A). Representative fluorescence images of gammaH2AX foci in serum starvation synchronized middle passage normal and HGPS fibroblasts after Dox treatment. Arrow points to the single gammaH2AX foci in the enlarged square. Scale Bar: 5um. (B). Line profile
Celeste Witting (3372776), Haoyue Zhang (3372770), Linlin Sun (396058), Di Wu (23906), Kan Cao (454493), Kun Wang (134525), Mason Trappio (3372773)   +6 more
core   +1 more source

INVESTIGATION OF ACCELERATED SKIN AGING AND PEROXISOMAL ABNORMALITIES IN HUTCHINSON-GILFORD PROGERIA SYNDROME [PDF]

, 2022
Hutchinson-Gilford progeria syndrome (HGPS) is a detrimental premature aging disease caused by a point mutation in human LMNA gene. This mutation results in the abnormal accumulation of a truncated pre-lamin A protein called progerin.
Mao, Xiaojing
core   +1 more source