Results 71 to 80 of about 2,950 (183)

Autophagic degradation of farnesylated prelamin A as a therapeutic approach to lamin-linked progeria

European Journal of Histochemistry, 2011
Farnesylated prelamin A is a processing intermediate produced in the lamin A maturation pathway. Accumulation of a truncated farnesylated prelamin A form, called progerin, is a hallmark of the severe premature ageing syndrome, Hutchinson-Gilford progeria.
V. Cenni, C. Capanni, M. Columbaro, M. Ortolani, M.R. D'Apice, G. Novelli, M. Fini, S. Marmiroli, E. Scarano, N.M. Maraldi, S. Squarzoni, S. Prencipe, G. Lattanzi   +12 more
doaj   +1 more source

Naïve adult stem cells from patients with Hutchinson-Gilford progeria syndrome express low levels of progerin in vivo

Biology Open, 2012
Summary Hutchinson-Gilford progeria syndrome (HGPS, OMIM 176670) is a rare disorder characterized by segmental accelerated aging and early death from coronary artery disease or stroke. Nearly 90% of HGPS sufferers carry a G608G mutation within exon 11 of
Vera Wenzel, Daniela Roedl, Diana Gabriel, Leslie B. Gordon, Meenhard Herlyn, Reinhard Schneider, Johannes Ring, Karima Djabali   +7 more
doaj   +1 more source

Cellular Senescence and Aging: Mechanisms, Disease Convergence, and Therapeutic Frontiers

MedComm, Volume 7, Issue 7, July 2026.
This schematic illustrates the hierarchical and interconnected nature of the primary molecular hallmarks of aging. The progression of aging is driven by a convergence of intrinsic molecular insults. Within the nucleus, genomic instability and telomere attrition trigger persistent DDR, accompanied by extensive epigenetic alterations.
Guowei Cai, Jia Ren, Li Wang, Yingying Wu, Huirui Wang, Xiaomeng Cao, Hao Shang, Xuben Hou, Yujiu Wang, Haibo Xue, Ting Dong   +10 more
wiley   +1 more source

Spatial changes of progerin expression in a neonatal DA.

, 2013
A. (b) Confocal images of immunofluorescence show even nuclear distribution of lamin A/C (red) and (c) nuclear envelope localization of progerin (green) in the media of DA. (a) Nuclei are counterstained with DAPI.
Regina Bökenkamp (344823), Elizabeth G. Nabel (75420), Michelle Olive (344826), Vered Raz (197916), Marco C. DeRuiter (344824), Andrea Venema (197914), Conny van Munsteren (344825), Adriana C. Gittenberger-de Groot (344827)   +7 more
core   +1 more source

Progerin in muscle leads to thermogenic and metabolic defects via impaired calcium homeostasis

, 2020
[[abstract]]Mutations in lamin A (LMNA) are responsible for a variety of human dystrophic and metabolic diseases. Here, we created a mouse model in which progerin, the lamin A mutant protein that causes Hutchinson-Gilford progeria syndrome (HGPS), can be
Wang, WP;Wang, JY;Lin, WH;Kao, CH;Hung, MC;Teng, YC;Tsai, TF;Chi, YH
core   +1 more source

Progerinin, an Inhibitor of Progerin, Alleviates Cardiac Abnormalities in a Model Mouse of Hutchinson–Gilford Progeria Syndrome

Cells, 2023
Hutchinson–Gilford Progeria Syndrome (HGPS) is an ultra-rare human premature aging disorder that precipitates death because of cardiac disease. Almost all cases of HGPS are caused by aberrant splicing of the LMNA gene that results in the production of a ...
So-mi Kang, Seungwoon Seo, Eun Ju Song, Okhee Kweon, Ah-hyeon Jo, Soyoung Park, Tae-Gyun Woo, Bae-Hoon Kim, Goo Taeg Oh, Bum-Joon Park   +9 more
doaj   +1 more source

Farnesyltransferase Deficiency in Cardiomyocytes Initiates Senescence and Contributes to Cardiac Fibrosis

Advanced Science, Volume 13, Issue 26, 8 May 2026.
Lipid overload suppresses SREBF2‐mediated FNTB expression, leading to defective Lamin A maturation and nuclear envelope instability. This nuclear catastrophe triggers a pro‐fibrotic senescence program in cardiomyocytes. Notably, restoring nuclear integrity via AAV9‐based gene therapy effectively attenuates cardiac remodeling, identifying the ...
Yuxiao Chen, Lian Lou, Xuan Zhang, Lin Yan, Qi Zhang, Wen Shi, Jie Ding, Xu Lin, Rong Jiang, Shuo Liu, Thida Sok, Pengli Wang, Yun Mou, Jie Han, Shenjiang Hu, Xiaogang Guo, Jian Yang   +16 more
wiley   +1 more source

Assessing the efficacy of protein farnesyltransferase inhibitors in mouse models of progeria

Journal of Lipid Research, 2010
Hutchinson-Gilford progeria syndrome (HGPS) is caused by the accumulation of a farnesylated form of prelamin A (progerin). Previously, we showed that blocking protein farnesylation with a farnesyltransferase inhibitor (FTI) ameliorates the disease ...
Shao H. Yang, Sandy Y. Chang, Douglas A. Andres, H. Peter Spielmann, Stephen G. Young, Loren G. Fong   +5 more
doaj   +1 more source

Advances in CRISPR Base Editing: From Molecular Evolution to Therapeutic Applications in Genomic Medicine

Journal of Cellular and Molecular Medicine, Volume 30, Issue 8, April 2026.
ABSTRACT CRISPR‐Cas9 systems revolutionized gene editing, but inherent drawbacks, namely DNA double‐strand breaks (DSBs) and the difficulty of achieving precise repairs (due to low HDR efficiency), led researchers to invent new, more accurate gene editing tools.
Melike Aliciaslan, Ezgi Erbasan, Fulya Erendor, Salih Sanlioglu   +3 more
wiley   +1 more source

Hematopoietic (stem) cells—The elixir of life?

FEBS Letters, Volume 600, Issue 4, Page 392-417, February 2026.
The aging of HSCs (hematopoietic stem cells) and the blood system leads to the decline of other organs. Rejuvenating aged HSCs improves the function of the blood system, slowing the aging of the heart, kidney, brain, and liver, and the occurrence of age‐related diseases.
Emilie L. Cerezo, Jonah Anderson, Emilie Dinh Vedrenne, Noël Yeh Martín, Jette Lengefeld   +4 more
wiley   +1 more source