Nuclear lamins and laminopathies

HJ Worman - The Journal of pathology, 2012 - Wiley Online Library
Nuclear lamins are intermediate filament proteins that polymerize to form the nuclear lamina
on the inner aspect of the inner nuclear membrane. Long known to be essential for …

“Laminopathies”: a wide spectrum of human diseases

HJ Worman, G Bonne - Experimental cell research, 2007 - Elsevier
Mutations in genes encoding the intermediate filament nuclear lamins and associated
proteins cause a wide spectrum of diseases sometimes called “laminopathies.” Diseases …

Lamin A/C deficiency causes defective nuclear mechanics and mechanotransduction

J Lammerding, PC Schulze… - The Journal of …, 2004 - Am Soc Clin Investig
Mutations in the lamin A/C gene (LMNA) cause a variety of human diseases including Emery-
Dreifuss muscular dystrophy, dilated cardiomyopathy, and Hutchinson-Gilford progeria …

Structural organization of nuclear lamins A, C, B1, and B2 revealed by superresolution microscopy

T Shimi, M Kittisopikul, J Tran… - Molecular biology of …, 2015 - Am Soc Cell Biol
The nuclear lamina is a key structural element of the metazoan nucleus. However, the
structural organization of the major proteins composing the lamina is poorly defined. Using …

Nuclear lamins: building blocks of nuclear architecture

RD Goldman, Y Gruenbaum, RD Moir… - Genes & …, 2002 - genesdev.cshlp.org
Nuclear lamins were initially identified as the major components of the nuclear lamina, a
proteinaceous layer found at the interface between chromatin and the inner nuclear …

Nuclear lamins: laminopathies and their role in premature ageing

JLV Broers, FCS Ramaekers, G Bonne… - Physiological …, 2006 - journals.physiology.org
It has been demonstrated that nuclear lamins are important proteins in maintaining cellular
as well as nuclear integrity, and in maintaining chromatin organization in the nucleus …

Concentric organization of A-and B-type lamins predicts their distinct roles in the spatial organization and stability of the nuclear lamina

B Nmezi, J Xu, R Fu, TJ Armiger… - Proceedings of the …, 2019 - National Acad Sciences
The nuclear lamina is an intermediate filament meshwork adjacent to the inner nuclear
membrane (INM) that plays a critical role in maintaining nuclear shape and regulating gene …

Lamin A/C expression is a marker of mouse and human embryonic stem cell differentiation

D Constantinescu, HL Gray, PJ Sammak… - Stem …, 2006 - academic.oup.com
Nuclear lamins comprise the nuclear lamina, a scaffold-like structure that lines the inner
nuclear membrane. B-type lamins are present in almost all cell types, but A-type lamins are …

Decreased mechanical stiffness in LMNA−/− cells is caused by defective nucleo-cytoskeletal integrity: implications for the development of laminopathies

JLV Broers, EAG Peeters, HJH Kuijpers… - Human molecular …, 2004 - academic.oup.com
Laminopathies comprise a group of inherited diseases with variable clinical phenotypes,
caused by mutations in the lamin A/C gene (LMNA). A prominent feature in several of these …

Lamins at a glance

CY Ho, J Lammerding - Journal of cell science, 2012 - journals.biologists.com
Lamins, the major components of the nuclear lamina, have gained rapidly increasing
interest over the past decade as lamin mutations were found to cause numerous devastating …