Results 31 to 40 of about 491 (128)

A multiparametric anti-aging CRISPR screen uncovers a role for BAF in protein synthesis regulation

Nature Communications
Progeria syndromes are very rare, incurable premature aging conditions recapitulating most aging features. Here, we report a whole genome, multiparametric CRISPR screen, identifying 43 genes that can rescue multiple cellular phenotypes associated with ...
Sophia Y. Breusegem, Jack Houghton, Raquel Romero-Bueno, Adrián Fragoso-Luna, Katherine A. Kentistou, Ken K. Ong, Anne F. J. Janssen, Nicholas A. Bright, Christian G. Riedel, John R. B. Perry, Peter Askjaer, Delphine Larrieu   +11 more
doaj   +1 more source

Targeting RANKL Prevents Bone Loss, Improves Muscle Function and Extends Lifespan in Progeroid Mice

Aging Cell, Volume 25, Issue 8, August 2026.
Targeting of RANKL by genetic and pharmacological approaches ameliorates key features of the progeroid phenotype in Zmpste24−/− mice. RANKL intervention restores bone mass, improves muscle phenotype, and extends survival. These findings support further exploration of RANKL‐targeted therapies for Hutchinson‐Gilford progeria syndrome.
Sandra Freitas‐Rodríguez, Alejandra Valle‐Cao, Francisco Rodríguez, Manuel Fernández‐Sanjurjo, Benjamín Fernández‐García, Vanessa Loredo, María Teresa Fernández‐García, Carmen Fiuza‐Luces, Alejandro Lucia, Carlos López‐Otín, Alejandro López‐Soto, Alicia R. Folgueras   +11 more
wiley   +1 more source

Progeria [PDF]

Саратовский научно-медицинский журнал, 2014
The data of literature, reflecting etiology, clinical features and differential diagnosis of progeria of childhood and adult are ...
Utz S.R., Talnikova Е.Е., Sherstneva V.N., Rumyantseva E.V..   +3 more
doaj  

Inhibition of the NLRP3 inflammasome improves lifespan in animal murine model of Hutchinson–Gilford Progeria

EMBO Molecular Medicine, 2021
Inflammation is a hallmark of aging and accelerated aging syndromes such as Hutchinson–Gilford progeria syndrome (HGPS). In this study, we present evidence of increased expression of the components of the NLRP3 inflammasome pathway in HGPS skin ...
Alvaro González‐Dominguez, Raúl Montañez, Beatriz Castejón‐Vega, Jéssica Nuñez‐Vasco, Débora Lendines‐Cordero, Chun Wang, Gabriel Mbalaviele, José M Navarro‐Pando, Elísabet Alcocer‐Gómez, Mario D Cordero   +9 more
doaj   +1 more source

Artery‐on‐Chip Demonstrates Mechanical and Functional Features of Healthy and Diseased Living Smooth Muscle Tissue

Advanced Functional Materials, Volume 36, Issue 59, 23 July 2026.
This article details the development of an artery‐on‐chip platform for in vitro arterial disease modeling and therapeutic discovery. It describes the fabrication of a fibrin biomaterial scaffold seeded with iPSC‐derived smooth muscle and endothelial cells, mimicking native artery properties. Two genetic disease models showcase the platform's ability to
Danielle Yarbrough, Roy Chen, Katerina Katsouleas, Jaron Shoemaker, Travis Brady, Tianfang Yu, Jennifer Arthur Ataam, Connor Amelung, Lakshmi Santhanam, Ioannis Karakikes, Sharon Gerecht   +10 more
wiley   +1 more source

Selection of specific and efficient siRNAs in new cellular model for Hutchinson-Gilford progeria syndrome therapy

Molecular Therapy: Nucleic Acids
Hutchinson-Gilford progeria syndrome is a fatal genetic disorder caused by a point mutation in the gene encoding the nuclear envelope protein lamin A/C.
Volha Dzianisava, Katarzyna Piekarowicz, Magdalena Machowska, Ryszard Rzepecki   +3 more
doaj   +1 more source

Multiscale Architecture and Mechanics of the Cell Nucleus: Implications for Disease, Bioengineering and Nanomedicine

Advanced Science, Volume 13, Issue 37, 3 July 2026.
Nuclear mechanical properties are inherently scale‐dependent, arising from a hierarchical architecture that spans DNA, chromatin, the nuclear envelope, and condensates. Experimental techniques and theoretical models are integrated into a cohesive multiscale framework linking nanoscale structural features to organelle‐level mechanical behavior.
Xinran Liu, Xin Chen, Qingyang Zhao, Weikang Tan, Lingrui Zhu, Kin Liao, Vincent Chan, Nicolae Goga, Winfred O. Larkotey, Yang Li, Yinghui Shi, Wenya Shu, Lin Wang, Weiqing Wang, Tian Jian Lu, Yulong Han   +15 more
wiley   +1 more source

Progeria

Indian journal of dermatology, venereology and leprology, 2012
A case of progeria is being reported in a 7-year old boy. He had characteristic facies, short stature, alopecia, high pitched voice, coxa valga and sclerodermatous changes in skin.
C, Kaur, S, Fatnani, A, Matravadia, K M, Acharya   +3 more
openaire   +3 more sources

Impact of Combined Baricitinib and FTI Treatment on Adipogenesis in Hutchinson–Gilford Progeria Syndrome and Other Lipodystrophic Laminopathies

Cells, 2023
Hutchinson–Gilford progeria syndrome (HGPS) is a rare genetic disease that causes premature aging symptoms, such as vascular diseases, lipodystrophy, loss of bone mineral density, and alopecia. HGPS is mostly linked to a heterozygous and de novo mutation
Ramona Hartinger, Eva-Maria Lederer, Elisa Schena, Giovanna Lattanzi, Karima Djabali   +4 more
doaj   +1 more source

Nuclear dysfunction in aging and neurodegeneration

Alzheimer's &Dementia, Volume 22, Issue 7, July 2026.
Abstract Neurodegenerative diseases are characterized by a loss of neuronal function and structure, often in a region‐specific manner. Multiple factors contribute to neuronal dysfunction and death, including pathogenic protein buildup, protein mislocalization, and inflammation. Despite extensive research, the common mechanisms driving neurodegeneration
Abbigael Aday, Jack Tiller, Adarsh Melukote, Zachary C. Walton, Anna King, Luke Castillo, Rakez Kayed, Mauro Montalbano   +7 more
wiley   +1 more source