Results 61 to 70 of about 17,639 (297)

Progerinin, an optimized progerin-lamin A binding inhibitor, ameliorates premature senescence phenotypes of Hutchinson-Gilford progeria syndrome

Communications Biology, 2021
Previous work has revealed that progerin-lamin A binding inhibitor (JH4) can ameliorate pathological features of Hutchinson-Gilford progeria syndrome (HGPS) such as nuclear deformation, growth suppression in patient’s cells, and very short life span in ...
So-mi Kang, Min-Ho Yoon, Jinsook Ahn, Ji-Eun Kim, So Young Kim, S. Kang, Jeongmin Joo, Soyoung Park, Jung-Hyun Cho, Tae-Gyun Woo, A. Oh, K. Chung, Solov'ev An, T. Hwang, Soo Yong Lee, Jeongsu Kim, N. Ha, G. Song, Bum-Joon Park   +18 more
semanticscholar   +1 more source

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

Vascular endothelium–targeted Sirt7 gene therapy rejuvenates blood vessels and extends life span in a Hutchinson-Gilford progeria model

Science Advances, 2020
Aged blood vessels accelerate systemic aging; SIRT7 gene therapy rejuvenates blood vessels and promotes longevity in aging model. Vascular dysfunction is a typical characteristic of aging, but its contributing roles to systemic aging and the therapeutic ...
Shimin Sun, Weifeng Qin, Xiaolong Tang, Yuan Meng, Wenjing Hu, Shuju Zhang, Minxian Qian, Zuojun Liu, Xinyue Cao, Qiuxiang Pang, Bosheng Zhao, Zimei Wang, Zhongjun Zhou, Baohua Liu   +13 more
semanticscholar   +1 more source

Nuclear membrane ruptures underlie the vascular pathology in a mouse model of Hutchinson-Gilford progeria syndrome

JCI Insight, 2021
The mutant nuclear lamin protein (progerin) produced in Hutchinson-Gilford progeria syndrome (HGPS) results in loss of arterial smooth muscle cells (SMCs), but the mechanism has been unclear. We found that progerin induces repetitive nuclear membrane (NM)
Paul H. Kim, Natalie Y. Chen, Patrick J. Heizer, Yiping Tu, Thomas A. Weston, Jared L.-C. Fong, N. Gill, A. Rowat, S. Young, L. Fong   +9 more
semanticscholar   +1 more source

Progeria - the old children

Quality in Sport
Introduction and Purpose: Progeria, also known as Hutchinson-Gilford progeria syndrome, is a rare genetic disorder characterized by accelerated aging in children.
Dawid Dziedziński, Dawid Łoś, Aleksandra Nowak, Anita Janus, Agata Kaptur   +4 more
doaj   +1 more source

Implant Supported Prosthesis in a Patient with Progeria: Case Report

Biomolecules & Biomedicine, 2009
Prosthodontic rehabilitation can be accomplished with fixed, overdenture, complete, or implant-retained prostheses. Dental treatment overcomes the patient’s functional, psychological, esthetic and phonation problems.
Gözlem Ceylan, Nergiz Yılmaz, Özgün Şenyurt, Göknil Ergün Kunt   +3 more
doaj   +1 more source

Alterations to nuclear architecture and genome behavior in senescent cells. [PDF]

, 2007
The organization of the genome within interphase nuclei, and how it interacts with nuclear structures is important for the regulation of nuclear functions.
Abney J.R., Agrelo R., Barboro P., Becker P.B., Bemiller P.M., Benn P.A., Berezney R., Bowman P.D., Bridger J.M., Bridger J.M., C. S. CLEMENTS, Campisi J., Chaly N., Chang K.S., Cremer T., de Lange T., Dell'Orco R.T., Dijkwel P.A., Ferbeyre G., Filesi I., Fisher A.G., Fleischer S., Goldman R.D., Gruenbaum Y., Haque F., Hernandez-Verdun D., Holliday R., Hozak P., I. R. KILL, I. S. MEHTA, J. M. BRIDGER, Kill I.R., Kreth G., Lezhava T., Li Y., M. FIGGITT, Mallette F.A., Manuelidis L., Mazin A.L., Mitsui Y., Mukherjee A.B., Nickerson J., Okabe J., Padiath Q.S., Pawelec G., Petrova N.V., Schardin M., Schulz L., Smilenov L.B., Strouboulis J., Tanabe H., van Driel R., Wang G.S.   +52 more
core   +1 more source

p300 nucleocytoplasmic shuttling underlies mTORC1 hyperactivation in Hutchinson–Gilford progeria syndrome

Nature Cell Biology
The mechanistic target of rapamycin complex 1 (mTORC1) is a master regulator of cell growth, metabolism and autophagy. Multiple pathways modulate mTORC1 in response to nutrients. Here we describe that nucleus–cytoplasmic shuttling of p300/EP300 regulates
Sung Min Son, So Jung Park, Sophia Y Breusegem, Delphine Larrieu, D. Rubinsztein   +4 more
semanticscholar   +1 more source

Mechanisms of angiogenic incompetence in Hutchinson–Gilford progeria via downregulation of endothelial NOS

Aging Cell, 2021
Hutchinson–Gilford progeria syndrome (HGPS) is a rare genetic disorder with features of accelerated aging. The majority of HGPS cases are caused by a de novo point mutation in the LMNA gene (c.1824C>T; p.G608G) resulting in progerin, a toxic lamin A ...
Yantenew G. Gete, Luke W. Koblan, Xiaojing Mao, Mason Trappio, B. Mahadik, J. Fisher, David R. Liu, Kan Cao   +7 more
semanticscholar   +1 more source

Dietary magnesium supplementation improves lifespan in a mouse model of progeria

EMBO Molecular Medicine, 2020
Aging is associated with redox imbalance according to the redox theory of aging. Consistently, a mouse model of premature aging (LmnaG609G/+) showed an increased level of mitochondrial reactive oxygen species (ROS) and a reduced basal antioxidant ...
Ricardo Villa‐Bellosta
doaj   +1 more source