Functional coupling between the extracellular matrix and nuclear lamina by Wnt signaling in progeria

L Hernandez, KJ Roux, ESM Wong, LC Mounkes… - Developmental cell, 2010 - cell.com
The segmental premature aging disease Hutchinson-Gilford Progeria (HGPS) is caused by
a truncated and farnesylated form of Lamin A. In a mouse model for HGPS, a similar Lamin A …

Telomere length in Hutchinson-Gilford progeria syndrome

ML Decker, E Chavez, I Vulto, PM Lansdorp - Mechanisms of ageing and …, 2009 - Elsevier
Hutchinson-Gilford Progeria Syndrome (HGPS) is a rare premature aging disorder caused
by mutations in the gene LMNA, which encodes the nuclear matrix protein lamin A. Previous …

Biomechanical strain exacerbates inflammation on a progeria‐on‐a‐chip model

J Ribas, YS Zhang, PR Pitrez, J Leijten, M Miscuglio… - small, 2017 - Wiley Online Library
Organ‐on‐a‐chip platforms seek to recapitulate the complex microenvironment of human
organs using miniaturized microfluidic devices. Besides modeling healthy organs, these …

Mechanisms of premature vascular aging in children with Hutchinson-Gilford progeria syndrome

M Gerhard-Herman, LB Smoot, N Wake, MW Kieran… - …, 2012 - Am Heart Assoc
Hutchinson-Gilford progeria syndrome is a rare, segmental premature aging syndrome of
accelerated atherosclerosis and early death from myocardial infarction or stroke. This study …

An overview of treatment strategies for Hutchinson-Gilford Progeria syndrome

K Harhouri, D Frankel, C Bartoli, P Roll… - Nucleus, 2018 - Taylor & Francis
Hutchinson-Gilford progeria syndrome (HGPS) is a sporadic, autosomal dominant disorder
characterized by premature and accelerated aging symptoms leading to death at the mean …

The posttranslational processing of prelamin A and disease

BSJ Davies, LG Fong, SH Yang… - Annual review of …, 2009 - annualreviews.org
Human geneticists have shown that some progeroid syndromes are caused by mutations
that interfere with the conversion of farnesyl-prelamin A to mature lamin A. For example …

Functional architecture of the cell's nucleus in development, aging, and disease

B Burke, CL Stewart - Current topics in developmental biology, 2014 - Elsevier
In eukaryotes, the function of the cell's nucleus has primarily been considered to be the
repository for the organism's genome. However, this rather simplistic view is undergoing a …

Cardiac abnormalities in patients with Hutchinson-Gilford progeria syndrome

A Prakash, LB Gordon, ME Kleinman… - JAMA …, 2018 - jamanetwork.com
Importance Hutchinson-Gilford progeria syndrome (HGPS) is an ultrarare disorder
associated with premature death due to cardiovascular events during the second decade of …

Loss of MTX2 causes mandibuloacral dysplasia and links mitochondrial dysfunction to altered nuclear morphology

S Elouej, K Harhouri, M Le Mao, G Baujat… - Nature …, 2020 - nature.com
Mandibuloacral dysplasia syndromes are mainly due to recessive LMNA or ZMPSTE24
mutations, with cardinal nuclear morphological abnormalities and dysfunction. We report five …

DNA damage: a main determinant of vascular aging

PK Bautista-Niño, E Portilla-Fernandez… - International journal of …, 2016 - mdpi.com
Vascular aging plays a central role in health problems and mortality in older people. Apart
from the impact of several classical cardiovascular risk factors on the vasculature …