Results 21 to 30 of about 4,296 (130)

The decision‐making process and criteria in selecting candidate drugs for progeria clinical trials

EMBO Molecular Medicine, 2016
Hutchinson–Gilford progeria syndrome (progeria) is an extremely rare premature aging disease with a population prevalence of 1 in 20 million. Nevertheless, propelled by the discovery of a causal mutation in the lamin A/C gene (LMNA) (De Sandre‐Giovannoli
Leslie B Gordon, Mark W Kieran, Monica E Kleinman, Tom Misteli   +3 more
doaj   +1 more source

Inhibition of DNA damage response at telomeres improves the detrimental phenotypes of Hutchinson–Gilford Progeria Syndrome

Nature Communications, 2019
Hutchinson–Gilford progeria syndrome causes premature aging. Here the authors show that activation of the DNA damage response at dysfunctional telomeres and transcription of telomeric non-coding RNAs contributes to the pathogenesis, which can be ...
Julio Aguado, Agustin Sola-Carvajal, Valeria Cancila, Gwladys Revêchon, Peh Fern Ong, Corey Winston Jones-Weinert, Emelie Wallén Arzt, Giovanna Lattanzi, Oliver Dreesen, Claudio Tripodo, Francesca Rossiello, Maria Eriksson, Fabrizio d’Adda di Fagagna   +12 more
doaj   +1 more source

Inhibiting Glutaminase Reverses Senescence‐Associated Mitochondrial Dysfunction and Skeletal Muscle Decline

Aging Cell, Volume 25, Issue 10, October 2026.
Pharmacological inhibition of glutaminase with drug CB‐839 restores mitochondrial function in senescent myoblasts and enhances muscle strength and endurance in progeria mice. ABSTRACT Age‐associated loss of skeletal muscle mass and function, also known as sarcopenia, is closely linked to mitochondrial dysfunction.
Hamsa Vardini Senthil Kumar, Debanik Choudhury, Pedro Lei, Pihu Mehrotra, Shahryar Shahini, Sydney Swedick, John Toftegaard, Stelios T. Andreadis   +7 more
wiley   +1 more source

Progeria in siblings: A rare case report

Indian Journal of Dermatology, 2011
Progeria, also known as Hutchinson-Gilford syndrome, is an extremely rare, severe genetic condition wherein symptoms resembling aspects of aging are manifested at an early age. It is an autosomal dominant disorder.
R Sowmiya, D Prabhavathy, S Jayakumar
doaj   +1 more source

Low and high expressing alleles of the LMNA gene: implications for laminopathy disease development. [PDF]

PLoS ONE, 2011
Today, there are at least a dozen different genetic disorders caused by mutations within the LMNA gene, and collectively, they are named laminopathies.
Sofía Rodríguez, Maria Eriksson
doaj   +1 more source

METTL14‐Mediated m6A Methylation Preserves Cardiomyocyte Homeostasis and Attenuates Progeria‐Associated Cardiac Remodeling

Aging Cell, Volume 25, Issue 10, October 2026.
Progerin accumulation downregulates the expression of m6A methyltransferases METTL3 and METTL14 in cardiac tissues, impairing m6A modification‐mediated regulation of target genes including Casq2, Ifna and Ifnb, leading to calcium signaling dysregulation and pro‐inflammatory activation in the aged heart.
Shixiao Wang, Jie Zhang, Ying Ao, Dan Rao, Cong Yu, Chenzhong Xu, Ming Wang, Mingying Li, Xin Huang, Jianfei Zhang, Rui Mei, Zhen Zhang, Jie Sun, Juanli Duan, Ying Sun, Zimei Wang, Wen Su, Baohua Liu   +17 more
wiley   +1 more source

MTR4: A Conserved Youth Factor for Vascular Endothelium

Aging Cell, Volume 25, Issue 10, October 2026.
Senescence‐induced SYVN1 degrades MTR4 via K638 ubiquitination. MTR4 loss disrupts its binding to BUB3 and destabilizes BUB3, diminishing BUBR1 and driving endothelial senescence. MTR4 restoration reverses vascular aging in mice and extends C. elegans lifespan.
Zhenyuan Guo, Zhiqiang Chen, Jiaqi Sun, Xiaojun Zhou, Chengzhi Ni, Yuxiang Zhang, Pengfei Qiang, Lu Zhang   +7 more
wiley   +1 more source

The Underexplored Mechanobiology of Lamin A Biogenesis and Homeostasis

Biology of the Cell, Volume 118, Issue 10, October 2026.
We highlight current evidence, unresolved questions, and future directions, proposing that mechanical regulation of lamin A biogenesis represents an underexplored dimension of lamin mechanobiology with broad implications for development, aging, laminopathies, and mechanically driven diseases. ABSTRACT Lamin A is a major contributor to nuclear mechanics
Sandrine Fraboulet, Omar Aldarawish, Monika Elzbieta Dolega   +2 more
wiley   +1 more source

Nucleic Acids as Emerging Regulators of Calcium Phosphate Biomineralization

The FASEB Journal, Volume 40, Issue 17, 15 September 2026.
Calcium phosphate biomineralization has traditionally been considered a protein‐regulated process. This review highlights the emerging role of nucleic acids, which interact with mineral phases through adsorption, coprecipitation, and templating, thereby influencing crystal nucleation and growth.
Fanny Duhalde, Jessem Landoulsi, Xavier Houard   +2 more
wiley   +1 more source

Hutchinson-Gilford progeria syndrome: a rare premature ageing syndrome

Przegląd Dermatologiczny, 2020
Hutchinson-Gilford progeria syndrome is an extremely rare genetic disorder characterized by premature ageing involving the skin, bones, heart, and blood vessels. The incidence is 1 in several million births.
Iti Varshney, Mohammad Adil, Syed Suhail Amin, Mohd Mohtashim, Annu Priya, Mahtab Alam   +5 more
doaj   +1 more source