[HTML][HTML] Activation of endoplasmic reticulum stress in premature aging via the inner nuclear membrane protein SUN2

S Vidak, LA Serebryannyy, G Pegoraro, T Misteli - Cell reports, 2023 - cell.com
One of the major cellular mechanisms to ensure cellular protein homeostasis is the
endoplasmic reticulum (ER) stress response. This pathway is triggered by accumulation of …

Protein sequestration at the nuclear periphery as a potential regulatory mechanism in premature aging

L Serebryannyy, T Misteli - Journal of Cell Biology, 2018 - rupress.org
Despite the extensive description of numerous molecular changes associated with aging,
insights into the driver mechanisms of this fundamental biological process are limited. Based …

Nuclear softening mediated by Sun2 suppression delays mechanical stress-induced cellular senescence

X Yue, J Cui, Z Sun, L Liu, Y Li, L Shao, Q Feng… - Cell Death …, 2023 - nature.com
Nuclear decoupling and softening are the main cellular mechanisms to resist mechanical
stress-induced nuclear/DNA damage, however, its molecular mechanisms remain much …

ESCRT-III controls nuclear envelope deformation induced by progerin

J Arii, F Maeda, Y Maruzuru, N Koyanagi, A Kato… - Scientific Reports, 2020 - nature.com
Hutchinson-Gilford progeria syndrome (HGPS) is a premature aging disorder, caused by
mutation in the gene encoding lamin A/C, which produces a truncated protein called …

Enhanced nuclear protein export in premature aging and rescue of the progeria phenotype by modulation of CRM1 activity

I García‐Aguirre, A Alamillo‐Iniesta… - Aging …, 2019 - Wiley Online Library
Abstract The study of Hutchinson–Gilford progeria syndrome (HGPS) has provided important
clues to decipher mechanisms underlying aging. Progerin, a mutant lamin A, disrupts …

Cytoskeleton stiffness regulates cellular senescence and innate immune response in Hutchinson–Gilford Progeria Syndrome

X Mu, C Tseng, WS Hambright, P Matre, CY Lin… - Aging …, 2020 - Wiley Online Library
Hutchinson–Gilford progeria syndrome (HGPS) is caused by the accumulation of mutant
prelamin A (progerin) in the nuclear lamina, resulting in increased nuclear stiffness and …

The defective nuclear lamina in Hutchinson-gilford progeria syndrome disrupts the nucleocytoplasmic Ran gradient and inhibits nuclear localization of Ubc9

JB Kelley, S Datta, CJ Snow, M Chatterjee… - … and Cellular Biology, 2011 - Am Soc Microbiol
The mutant form of lamin A responsible for the premature aging disease Hutchinson-Gilford
progeria syndrome (termed progerin) acts as a dominant negative protein that changes the …

A pathway linking oxidative stress and the Ran GTPase system in progeria

S Datta, CJ Snow, BM Paschal - Molecular biology of the cell, 2014 - Am Soc Cell Biol
Maintaining the Ran GTPase at a proper concentration in the nucleus is important for
nucleocytoplasmic transport. Previously we found that nuclear levels of Ran are reduced in …

Imbalanced nucleocytoskeletal connections create common polarity defects in progeria and physiological aging

W Chang, Y Wang, GWG Luxton… - Proceedings of the …, 2019 - National Acad Sciences
Studies of the accelerated aging disorder Hutchinson–Gilford progeria syndrome (HGPS)
can potentially reveal cellular defects associated with physiological aging. HGPS results …

Nucleolar expansion and elevated protein translation in premature aging

A Buchwalter, MW Hetzer - Nature communications, 2017 - nature.com
Premature aging disorders provide an opportunity to study the mechanisms that drive aging.
In Hutchinson-Gilford progeria syndrome (HGPS), a mutant form of the nuclear scaffold …