Results 61 to 70 of about 4,130 (205)

Temsirolimus does not impact DNA damage levels in HGPS cells.

, 2016
(A) Immunohistochemistry of control (GMO3349C) and HGPS (HGPSFN003) cells either mock-treated or treated with 1.0 μM temsirolimus for a period of 9 days. Antibodies against γH2A.X and 53BP1 were used (n = 4). Scale bar: 20 μm. (B) Immunohistochemistry of
Diana Gabriel (3608093), Karima Djabali (82182), Leslie B. Gordon (82180)   +2 more
core   +1 more source

Histidine Supplementation Stabilizes Hearing and Vision and Improves Growth in HARS1‐Related Autosomal Recessive Disorder Associated With Usher‐Like Symptoms

American Journal of Medical Genetics Part A, Volume 200, Issue 10, Page 2289-2308, October 2026.
ABSTRACT Autosomal recessive HARS1‐related disorder (originally described as Usher syndrome type 3B) caused by a homozygous Y454S variant in the histidyl‐tRNA synthetase gene (HARS1) is characterized by progressive sensorineural hearing and vision loss and respiratory deterioration with risk for sudden death following febrile illnesses.
Victoria Mok Siu, Rosan Kenana, Rana Chakrabarti, Sarah D. P. Wilhelm, Joseph Andrews, Susan J. Leat, Christina Parker, Michael Miller, Leslie A. Nangle, Wendy McCaul, Ashfia Chowdhury, Natalie Hutchings, Ryan A. Adams, Lauren Guy, Mandy Rhody, Verena Juncal, Marisa I. Mendes, Desiree E. C. Smith, Gajja S. Salomons, Angelica A. Moresco, Daphne L. McCulloch, D. Holmes Morton, Ilka U. Heinemann, C. Anthony Rupar   +23 more
wiley   +1 more source

LivAge: An Online Aging Clock for Murine Transcriptomic Age Estimation

Aging Cell, Volume 25, Issue 9, September 2026.
We present LivAge, an online aging clock that estimates murine transcriptomic age from hepatic RNA‐seq data. Externally validated, it detects accelerated aging in progeroid models and quantifies the effect of geroprotective interventions, providing an accurate, accessible, and ready‐to‐use tool for aging research.
Víctor Celemín‐Capaldi, Guillermina Bea, David Roiz‐Valle, Alejandro P. Ugalde, Clea Bárcena, Pedro M. Quirós, José M. P. Freije   +6 more
wiley   +1 more source

SerpinE1 drives a cell-autonomous pathogenic signaling in Hutchinson–Gilford progeria syndrome

Cell Death and Disease, 2022
Hutchinson–Gilford progeria syndrome (HGPS) is a rare, fatal disease caused by Lamin A mutation, leading to altered nuclear architecture, loss of peripheral heterochromatin and deregulated gene expression.
Giorgia Catarinella, Chiara Nicoletti, Andrea Bracaglia, Paola Procopio, Illari Salvatori, Marilena Taggi, Cristiana Valle, Alberto Ferri, Rita Canipari, Pier Lorenzo Puri, Lucia Latella   +10 more
doaj   +1 more source

Akkermansia muciniphila and Its Bioactive Derivatives: Emerging Regulators of Healthy Aging

Aging Cell, Volume 25, Issue 9, September 2026.
The beneficial gut bacterium Akkermansia muciniphila (AKK) declines with age but is enriched in healthy centenarians. Supplementation of AKK extends healthspan and alleviates age‐related decline through direct host–microbe interactions and its bioactive derivatives, which collectively enhance gut barrier integrity, restore metabolic homeostasis ...
Ting Zhang, Jinyuan Niu, Xiaoyu Hu, Zhiang Hu, Yuan Gao, Jialiang Liu, Ning Chen, Yaomin Hu   +7 more
wiley   +1 more source

De HGP-write aux « super-cellules » [PDF]

médecine/sciences, 2018
The HGP-write project, announced in 2016 but not really implemented yet, comes back as a project aimed at constructing an “ultra-safe” human cell line fully resistant to virus infection and with other desirable characteristics. This involves introducing 400,000 changes in the genome and raises a number of technical and financial issues, but may become ...
openaire   +2 more sources

Proteostasis of organelles in aging and disease

The FEBS Journal, Volume 293, Issue 18, Page 5600-5626, September 2026.
Cells rely on regulated proteostasis mechanisms to keep their internal compartments functioning properly. When these mechanisms fail, damaged proteins accumulate, disrupting organelles, such as the nucleus, mitochondria, endoplasmic reticulum, Golgi, and lysosomes, as well as membraneless organelles, such as stress granules, processing bodies, the ...
Yara Nabawi, Cansu Doğan, Dunja Petrović, Gülce Perçin, Seda Koyuncu, David Vilchez   +5 more
wiley   +1 more source

gammaH2AX signal reduction was caused by defective ATM activation in HGPS.

, 2016
(A). (upper): Western blotting analysis with anti-pATM(S1981), anti-ATM and anti-GAPDH antibodies on serum starvation synchronized middle passage normal and HGPS fibroblasts with or without Dox treatment.
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

Transforming Life Science Through Chromosome‐Level Genome Assemblies

Genes to Cells, Volume 31, Issue 5, September 2026.
Chromosome‐level genome assemblies provide a framework for “glocal biology” in species of interest, linking local molecular features to chromosome architecture and evolutionary context. When integrated with pangenome analysis, comparative multi‐omics, genome language models, and experimental approaches, these assemblies enable hypothesis generation and
Tetsuo Kon, Kosuke Kataoka, Yi‐Jyun Luo, Johannes Nicolaus Wibisana, Kouhei Toga, Narumi Uno, Yuki Monden, Mayuko Hamada   +7 more
wiley   +1 more source

Vascular cells derived from Hutchinson-Gilford progeria syndrome (HGPS) inducible pluripotent stem cells [PDF]

, 2014
To study the vulnerability of smooth muscle cells (SMCs) in Hutchinson-Gilford Progeria Syndrome (HGPS)
Rocha, I., Ferreira, Lino, Nissan, Xavier, Vazão, Helena, Pitrez, Patricia R.   +4 more
core   +2 more sources