Results 11 to 20 of about 17,639 (297)

Progeria and Aging—Omics Based Comparative Analysis

Biomedicines, 2022
Since ancient times aging has also been regarded as a disease, and humankind has always strived to extend the natural lifespan. Analyzing the genes involved in aging and disease allows for finding important indicators and biological markers for ...
Aylin Caliskan, Samantha A. W. Crouch, Sara Giddins, Thomas Dandekar, Seema Dangwal   +4 more
doaj   +2 more sources

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

Nature Communications, 2019
Hutchinson–Gilford progeria syndrome (HGPS) is a genetic disorder characterized by premature aging features. Cells from HGPS patients express progerin, a truncated form of Lamin A, which perturbs cellular homeostasis leading to nuclear shape alterations,
Aguado J., Cancila V., d'Adda di Fagagna F., Dreesen O., Eriksson M., Jones-Weinert C. W., Lattanzi G., Ong P. F., Revechon G., Rossiello F., Sola-Carvajal A., Tripodo C., Wallen Arzt E.   +12 more
core   +2 more sources

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   +2 more sources

Defining the progeria phenome. [PDF]

Aging (Albany NY)
Progeroid disorders are a heterogenous group of rare and complex hereditary syndromes presenting with pleiotropic phenotypes associated with normal aging. Due to the large variation in clinical presentation the diseases pose a diagnostic challenge for clinicians which consequently restricts medical research.
Worm C, Schambye MER, Mkrtchyan GV, Veviorskiy A, Shneyderman A, Ozerov IV, Zhavoronkov A, Bakula D, Scheibye-Knudsen M.   +8 more
europepmc   +5 more sources

The farnesyl transferase inhibitor (FTI) lonafarnib improves nuclear morphology in ZMPSTE24-deficient fibroblasts from patients with the progeroid disorder MAD-B

Nucleus, 2023
Several related progeroid disorders are caused by defective post-translational processing of prelamin A, the precursor of the nuclear scaffold protein lamin A, encoded by LMNA.
Kamsi O. Odinammadu, Khurts Shilagardi, Kelsey Tuminelli, Daniel P. Judge, Leslie B. Gordon, Susan Michaelis   +5 more
doaj   +1 more source

Progeria Presenting with Pyogenic Granuloma in Conjunctiva: A Case Report

Journal of Nepal Medical Association, 2023
Hutchinson-Gilford progeria syndrome frequently exhibits stunted growth and premature ageing. Notable ocular characteristics can encompass a large number of ocular abnormalities.
Sanket Parajuli, Sadhana Sharma, Tina Shrestha   +2 more
doaj   +1 more source

The Senolytic Drug Fisetin Attenuates Bone Degeneration in the Zmpste24−/− Progeria Mouse Model

Journal of Osteoporosis, 2023
Aging leads to several geriatric conditions including osteoporosis (OP) and associated frailty syndrome. Treatments for these conditions are limited and none target fundamental drivers of pathology, and thus identifying strategies to delay progressive ...
W. S. Hambright, Xiaodong Mu, Xueqin Gao, Ping Guo, Y. Kawakami, J. Mitchell, M. Mullen, A. Nelson, Chelsea S. Bahney, H. Nishimura, Justin E. Hellwinkel, Andrew Eck, J. Huard   +12 more
semanticscholar   +1 more source

Hereditary syndromes with signs of premature aging [PDF]

Остеопороз и остеопатии, 2020
Aging is a multi-factor biological process that inevitably affects everyone. Degenerative processes, starting at the cellular and molecular levels, gradually influence the change in the functional capabilities of all organs and systems.
Olga O. Golounina, Valentin V. Fadeev, Zhanna E. Belaya   +2 more
doaj   +1 more source

Transcriptional activation of endogenous Oct4 via the CRISPR/dCas9 activator ameliorates Hutchinson‐Gilford progeria syndrome in mice

Aging Cell, 2023
Partial cellular reprogramming via transient expression of Oct4, Sox2, Klf4, and c‐Myc induces rejuvenation and reduces aged‐cell phenotypes. In this study, we found that transcriptional activation of the endogenous Oct4 gene by using the CRISPR/dCas9 ...
Junyeop Kim, Yerim Hwang, Sumin Kim, Yujung Chang, Yunkyung Kim, Youngeun Kwon, Jongpil Kim   +6 more
semanticscholar   +1 more source

Unique progerin C-terminal peptide ameliorates Hutchinson–Gilford progeria syndrome phenotype by rescuing BUBR1

Nature Aging, 2023
An accumulating body of evidence indicates an association between mitotic defects and the aging process in Hutchinson–Gilford progeria syndrome (HGPS), which is a premature aging disease caused by progerin accumulation.
Na Zhang, Qianying Hu, Tingting Sui, Lu Fu, Xinglin Zhang, Y. Wang, Xiaojuan Zhu, Baiqu Huang, Jun Lu, Zhanjun Li, Yu Zhang   +10 more
semanticscholar   +1 more source