A cell-intrinsic interferon-like response links replication stress to cellular aging caused by progerin

R Kreienkamp, S Graziano, N Coll-Bonfill… - Cell reports, 2018 - cell.com
Hutchinson-Gilford progeria syndrome (HGPS) is a premature aging disease caused by a
truncated lamin A protein (progerin) that drives cellular and organismal decline. HGPS …

Rapamycin reverses cellular phenotypes and enhances mutant protein clearance in Hutchinson-Gilford progeria syndrome cells

K Cao, JJ Graziotto, CD Blair, JR Mazzulli… - Science translational …, 2011 - science.org
Hutchinson-Gilford progeria syndrome (HGPS) is a lethal genetic disorder characterized by
premature aging. HGPS is most commonly caused by a de novo single-nucleotide …

[HTML][HTML] Vitamin D receptor signaling improves Hutchinson-Gilford progeria syndrome cellular phenotypes

R Kreienkamp, M Croke, MA Neumann, G Bedia-Diaz… - Oncotarget, 2016 - ncbi.nlm.nih.gov
Abstract Hutchinson-Gilford Progeria Syndrome (HGPS) is a devastating incurable
premature aging disease caused by accumulation of progerin, a toxic lamin A mutant …

Repression of the antioxidant NRF2 pathway in premature aging

N Kubben, W Zhang, L Wang, TC Voss, J Yang, J Qu… - Cell, 2016 - cell.com
Hutchinson-Gilford progeria syndrome (HGPS) is a rare, invariably fatal premature aging
disorder. The disease is caused by constitutive production of progerin, a mutant form of the …

Progerin-induced replication stress facilitates premature senescence in Hutchinson-Gilford progeria syndrome

K Wheaton, D Campuzano, W Ma… - … and cellular biology, 2017 - Taylor & Francis
Hutchinson-Gilford progeria syndrome (HGPS) is caused by a mutation in LMNA that
produces an aberrant lamin A protein, progerin. The accumulation of progerin in HGPS cells …

Interruption of progerin–lamin A/C binding ameliorates Hutchinson-Gilford progeria syndrome phenotype

SJ Lee, YS Jung, MH Yoon, S Kang… - The Journal of …, 2016 - Am Soc Clin Investig
Hutchinson-Gilford progeria syndrome (HGPS) is a rare autosomal dominant genetic
disease that is caused by a silent mutation of the LMNA gene encoding lamins A and C …

Hutchinson-Gilford Progeria Syndrome: A premature aging disease caused by LMNA gene mutations

S Gonzalo, R Kreienkamp, P Askjaer - Ageing research reviews, 2017 - Elsevier
Products of the LMNA gene, primarily lamin A and C, are key components of the nuclear
lamina, a proteinaceous meshwork that underlies the inner nuclear membrane and is …

Hutchinson–Gilford progeria syndrome through the lens of transcription

M Prokocimer, R Barkan, Y Gruenbaum - Aging Cell, 2013 - Wiley Online Library
Lamins are nuclear intermediate filaments. In addition to their structural roles, they are
implicated in basic nuclear functions such as chromatin organization, DNA replication …

Lamin A-dependent misregulation of adult stem cells associated with accelerated ageing

P Scaffidi, T Misteli - Nature cell biology, 2008 - nature.com
The premature-ageing disease Hutchinson-Gilford Progeria Syndrome (HGPS) is caused by
constitutive production of progerin, a mutant form of the nuclear architectural protein lamin …

[HTML][HTML] Hutchinson-Gilford progeria syndrome: Cellular mechanisms and therapeutic perspectives

B Cisneros, I García-Aguirre, M De Ita… - Archives of Medical …, 2023 - Elsevier
In humans, aging is characterized by a gradual decline of physical and psychological
functions, with the concomitant onset of chronic-degenerative diseases, which ultimately …