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 …

Coaching from the sidelines: the nuclear periphery in genome regulation

A Buchwalter, JM Kaneshiro, MW Hetzer - Nature Reviews Genetics, 2019 - nature.com
The genome is packaged and organized nonrandomly within the 3D space of the nucleus to
promote efficient gene expression and to faithfully maintain silencing of heterochromatin …

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 …

A-type lamins bind both hetero-and euchromatin, the latter being regulated by lamina-associated polypeptide 2 alpha

K Gesson, P Rescheneder, MP Skoruppa… - Genome …, 2016 - genome.cshlp.org
Lamins are components of the peripheral nuclear lamina and interact with heterochromatic
genomic regions, termed lamina-associated domains (LADs). In contrast to lamin B1 being …

Lamins in the nuclear interior− life outside the lamina

N Naetar, S Ferraioli, R Foisner - Journal of cell science, 2017 - journals.biologists.com
Nuclear lamins are components of the peripheral lamina that define the mechanical
properties of nuclei and tether heterochromatin to the periphery. A-type lamins localize also …

Telomere-associated aging disorders

PL Opresko, JW Shay - Ageing research reviews, 2017 - Elsevier
Telomeres are dynamic nucleoprotein-DNA structures that cap and protect linear
chromosome ends. Several monogenic inherited diseases that display features of human …

Nuclear envelope dysfunction and its contribution to the aging process

F Martins, J Sousa, CD Pereira… - Aging cell, 2020 - Wiley Online Library
The nuclear envelope (NE) is the central organizing unit of the eukaryotic cell serving as a
genome protective barrier and mechanotransduction interface between the cytoplasm and …

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 …

The histone code of senescence

H Paluvai, E Di Giorgio, C Brancolini - Cells, 2020 - mdpi.com
Senescence is the end point of a complex cellular response that proceeds through a set of
highly regulated steps. Initially, the permanent cell-cycle arrest that characterizes …

The nuclear lamina in health and disease

A Dobrzynska, S Gonzalo, C Shanahan, P Askjaer - Nucleus, 2016 - Taylor & Francis
The nuclear lamina (NL) is a structural component of the nuclear envelope and makes
extensive contacts with integral nuclear membrane proteins and chromatin. These …