Intermediate filament (IF) proteins and heat shock proteins (HSPs) are large multimember
families that share several features, including protein abundance, significant upregulation in …

Mechanotransduction translates forces into biological responses and regulates cell
functionalities. It is implicated in several diseases, including laminopathies which are …

The nuclear lamina provides structural support to the nucleus and has a central role in
nuclear organization and gene regulation. Defects in its constituents, the lamins, lead to a …

Fibroblasts from patients with the severe laminopathy diseases, restrictive dermopathy (RD)
and Hutchinson Gilford progeria syndrome (HGPS), are characterized by poor growth in …

Mutations in the human LMNA gene cause muscular dystrophy by mechanisms that are
incompletely understood. The LMNA gene encodes A-type lamins, intermediate filaments …

The nuclear lamina contributes to the regulation of gene expression and to chromatin
organization. Mutations in A-type nuclear lamins cause laminopathies, some of which are …

Laminopathies are a clinically heterogeneous group of disorders caused by mutations in
LMNA. The main proteins encoded by LMNA are Lamin A and C, which together with Lamin …

Lamins are the main component of the nuclear lamina, a protein meshwork at the inner
nuclear membrane which primarily provide mechanical stability to the nucleus. Lamins, type …

Several alterations in nuclear envelope proteins building up the lamina meshwork beneath
the inner nuclear membrane (mutations in lamins A/C, alterations of prelamin-A maturation …

Mutations in LMNA cause a variety of diseases affecting striated muscle including autosomal
Emery‐Dreifuss muscular dystrophy (EDMD), LMNA‐associated congenital muscular …