Diverse lamin-dependent mechanisms interact to control chromatin dynamics: Focus on laminopathies
Academic Article
Publication Date:
2014
abstract:
Interconnected functional strategies govern chromatin dynamics in eukaryotic cells. In this context, Aand B type lamins, the nuclear intermediate filaments, act on diverse platforms involved in tissue homeostasis. On the nuclear side, lamins elicit large scale or fine chromatin conformational changes, affect DNAdamage response factors and transcription factor shuttling. On the cytoplasmic side, bridging-molecules, the LINC complex, associate with lamins to coordinate chromatin dynamics with cytoskeleton and extra-cellular signals.,Consistent with such a fine tuning, lamin mutations and/or defects in their expression or post-translational processing, as well as mutations in lamin partner genes, cause a heterogeneous group of diseases known as laminopathies. They include muscular dystrophies, cardiomyopathy, lipodystrophies, neuropathies, and progeroid syndromes. The study of chromatin dynamics under pathological conditions, which is summarized in this review, is shedding light on the complex and fascinating role of the nuclear lamina in chromatin regulation.
Iris type:
01.01 Articolo in rivista
Keywords:
Chromatin; lamin; Emery-Dreifuss muscular dystrophy (EDMD); Familial partial lipodystrophy (FPLD2); Hutchinson-Gilford progeria (HGPS); LADs; Laminopathies; Lamins; Mandibuloacral dysplasia; Nuclear envelope proteins
List of contributors:
Lattanzi, Giovanna; Capanni, Cristina; Cenni, Vittoria; Mattioli, Elisabetta; Squarzoni, Stefano
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