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.

Diverse lamin-dependent mechanisms interact to control chromatin dynamics: Focus on laminopathies

Capanni C;Cenni V;Mattioli E;Squarzoni S;Lattanzi G
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.
2014
Istituto di Genetica Molecolare "Luigi Luca Cavalli Sforza"
Chromatin
lamin
Emery-Dreifuss muscular dystrophy (EDMD)
Familial partial lipodystrophy (FPLD2)
Hutchinson-Gilford progeria (HGPS)
LADs
Laminopathies
Lamins
Mandibuloacral dysplasia
Nuclear envelope proteins
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Utilizza questo identificativo per citare o creare un link a questo documento: https://hdl.handle.net/20.500.14243/225684
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