Hutchinson-gilford progeria syndrome: challenges at bench and bedside

R Kreienkamp, S Gonzalo - Biochemistry and Cell Biology of Ageing: Part II …, 2019 - Springer
The structural nuclear proteins known as “lamins”(A-type and B-type) provide a scaffold for
the compartmentalization of genome function that is important to maintain genome stability …

[HTML][HTML] Hutchinson-Gilford progeria syndrome: a literature review

A Lamis, SW Siddiqui, T Ashok, N Patni, M Fatima… - Cureus, 2022 - ncbi.nlm.nih.gov
Hutchinson-Gilford progeria syndrome (HGPS) is a premature aging condition that involves
genetic mutations, resulting in debilitating phenotypic features. The present state of …

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 …

Genomic instability and innate immune responses to self-DNA in progeria

S Gonzalo, N Coll-Bonfill - GeroScience, 2019 - Springer
In the last decade, we have seen increasing evidence of the importance of structural nuclear
proteins such as lamins in nuclear architecture and compartmentalization of genome …

[HTML][HTML] Molecular insights into the premature aging disease progeria

S Vidak, R Foisner - Histochemistry and cell biology, 2016 - Springer
Hutchinson–Gilford progeria syndrome (HGPS) is an extremely rare premature aging
disease presenting many features resembling the normal aging process. HGPS patients die …

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 …

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] 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 …

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 …

Towards delineating the chain of events that cause premature senescence in the accelerated aging syndrome Hutchinson–Gilford progeria (HGPS)

O Dreesen - Biochemical Society Transactions, 2020 - portlandpress.com
The metazoan nucleus is equipped with a meshwork of intermediate filament proteins called
the A-and B-type lamins. Lamins lie beneath the inner nuclear membrane and serve as a …