Back to Search
Start Over
Lamin A/C Mechanotransduction in Laminopathies
- Source :
- Cells, Cells, Vol 9, Iss 1306, p 1306 (2020)
- Publication Year :
- 2020
-
Abstract
- Mechanotransduction translates forces into biological responses and regulates cell functionalities. It is implicated in several diseases, including laminopathies which are pathologies associated with mutations in lamins and lamin-associated proteins. These pathologies affect muscle, adipose, bone, nerve, and skin cells and range from muscular dystrophies to accelerated aging. Although the exact mechanisms governing laminopathies and gene expression are still not clear, a strong correlation has been found between cell functionality and nuclear behavior. New theories base on the direct effect of external force on the genome, which is indeed sensitive to the force transduced by the nuclear lamina. Nuclear lamina performs two essential functions in mechanotransduction pathway modulating the nuclear stiffness and governing the chromatin remodeling. Indeed, A-type lamin mutation and deregulation has been found to affect the nuclear response, altering several downstream cellular processes such as mitosis, chromatin organization, DNA replication-transcription, and nuclear structural integrity. In this review, we summarize the recent findings on the molecular composition and architecture of the nuclear lamina, its role in healthy cells and disease regulation. We focus on A-type lamins since this protein family is the most involved in mechanotransduction and laminopathies.
- Subjects :
- Emery-Dreyfuss muscular dystrophy
Hutchinson Gilford progeria syndrome
gene regulation
lamin A/C
lamin partners
laminopathy
mechanotransduction
Laminopathy
Review
Biology
Mechanotransduction, Cellular
Chromatin remodeling
03 medical and health sciences
0302 clinical medicine
medicine
Animals
Humans
Mechanotransduction
Mitosis
lcsh:QH301-705.5
030304 developmental biology
Regulation of gene expression
0303 health sciences
Laminopathies
General Medicine
medicine.disease
Lamin Type A
Chromatin
Cell biology
lcsh:Biology (General)
Mutation
Nuclear lamina
030217 neurology & neurosurgery
Lamin
Protein Binding
Subjects
Details
- Language :
- English
- Database :
- OpenAIRE
- Journal :
- Cells, Cells, Vol 9, Iss 1306, p 1306 (2020)
- Accession number :
- edsair.doi.dedup.....c330e87980ab0ef77345067136eed310