Phosphorylated lamin A/C in the nuclear interior binds active enhancers associated with abnormal transcription in progeria

K Ikegami, S Secchia, O Almakki, JD Lieb… - Developmental cell, 2020 - cell.com
LMNA encodes nuclear Lamin A/C that tethers lamina-associated domains (LADs) to the
nuclear periphery. Mutations in LMNA cause degenerative disorders including the …

Dysregulated interactions between lamin A and SUN1 induce abnormalities in the nuclear envelope and endoplasmic reticulum in progeric laminopathies

ZJ Chen, WP Wang, YC Chen, JY Wang… - Journal of cell …, 2014 - journals.biologists.com
Hutchinson-Gilford progeria syndrome (HGPS) is a human progeroid disease caused by a
point mutation on the LMNA gene. We reported previously that the accumulation of the …

Accumulation of mutant lamin A causes progressive changes in nuclear architecture in Hutchinson–Gilford progeria syndrome

RD Goldman, DK Shumaker… - Proceedings of the …, 2004 - National Acad Sciences
Hutchinson–Gilford progeria syndrome (HGPS) is a premature aging disorder, commonly
caused by a point mutation in the lamin A gene that results in a protein lacking 50 aa near …

Mapping of lamin A-and progerin-interacting genome regions

N Kubben, M Adriaens, W Meuleman, JW Voncken… - Chromosoma, 2012 - Springer
Mutations in the A-type lamins A and C, two major components of the nuclear lamina, cause
a large group of phenotypically diverse diseases collectively referred to as laminopathies …

Aging and nuclear organization: lamins and progeria

LC Mounkes, CL Stewart - Current opinion in cell biology, 2004 - Elsevier
The discoveries of at least eight human diseases arising from mutations in LMNA, which
encodes the nuclear A-type lamins, have revealed the nuclear envelope as an organelle …

A progeria mutation reveals functions for lamin A in nuclear assembly, architecture, and chromosome organization

P Taimen, K Pfleghaar, T Shimi… - Proceedings of the …, 2009 - National Acad Sciences
Numerous mutations in the human A-type lamin gene (LMNA) cause the premature aging
disease, progeria. Some of these are located in the αあるふぁ-helical central rod domain required for …

Proliferation of progeria cells is enhanced by lamina-associated polypeptide 2αあるふぁ (LAP2αあるふぁ) through expression of extracellular matrix proteins

S Vidak, N Kubben, T Dechat… - Genes & …, 2015 - genesdev.cshlp.org
Lamina-associated polypeptide 2αあるふぁ (LAP2αあるふぁ) localizes throughout the nucleoplasm and
interacts with the fraction of lamins A/C that is not associated with the peripheral nuclear …

Distinct structural and mechanical properties of the nuclear lamina in Hutchinson–Gilford progeria syndrome

KN Dahl, P Scaffidi, MF Islam… - Proceedings of the …, 2006 - National Acad Sciences
The nuclear lamina is a network of structural filaments, the A and B type lamins, located at
the nuclear envelope and throughout the nucleus. Lamin filaments provide the nucleus with …

Role of A-type lamins in signaling, transcription, and chromatin organization

V Andrés, JM González - Journal of Cell Biology, 2009 - rupress.org
A-type lamins (lamins A and C), encoded by the LMNA gene, are major protein constituents
of the mammalian nuclear lamina, a complex structure that acts as a scaffold for protein …

Alterations in mitosis and cell cycle progression caused by a mutant lamin A known to accelerate human aging

T Dechat, T Shimi, SA Adam… - Proceedings of the …, 2007 - National Acad Sciences
Mutations in the gene encoding nuclear lamin A (LA) cause the premature aging disease
Hutchinson–Gilford Progeria Syndrome. The most common of these mutations results in the …