title: Diverse lamin-dependent mechanisms interact to control chromatin dynamics. Focus on laminopathies.
abstract: Interconnected functional strategies govern chromatin dynamics in eukaryotic cells. In this context, A and 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 DNA damage 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.
authors.affiliation: a CNR Institute for Molecular Genetics; Unit of Bologna and SC Laboratory of Musculoskeletal Cell Biology; Rizzoli Orthopedic Institute; Bologna, Italy.
authors.forename: Daria
authors.initials: D
authors.lastname: Camozzi
authors.forename: Cristina
authors.initials: C
authors.lastname: Capanni
authors.forename: Vittoria
authors.initials: V
authors.lastname: Cenni
authors.forename: Elisabetta
authors.initials: E
authors.lastname: Mattioli
authors.forename: Marta
authors.initials: M
authors.lastname: Columbaro
authors.forename: Stefano
authors.initials: S
authors.lastname: Squarzoni
authors.forename: Giovanna
authors.initials: G
authors.lastname: Lattanzi
mesh_terms: D030981:Acro-Osteolysis / Q000235:genetics / Q000473:pathology; D002843:Chromatin / Q000235:genetics*; D022004:Chromosome Structures / Q000235:genetics; D003599:Cytoskeleton / Q000235:genetics*; D006801:Humans; D034904:Lamin Type A / Q000235:genetics*; D034921:Lamin Type B / Q000235:genetics*; D008060:Lipodystrophy / Q000235:genetics / Q000473:pathology; D052496:Lipodystrophy, Familial Partial / Q000235:genetics / Q000473:pathology; D008334:Mandible / Q000002:abnormalities / Q000473:pathology; D020389:Muscular Dystrophy, Emery-Dreifuss / Q000235:genetics / Q000473:pathology; D009154:Mutation; D009685:Nuclear Envelope / Q000235:genetics; D011371:Progeria / Q000235:genetics / Q000473:pathology
chemical_list: D002843:Chromatin; D034904:Lamin Type A; D034921:Lamin Type B
keywords: Emery-Dreifuss muscular dystrophy; Hutchinson-Gilford progeria; LADs; chromatin; familial partial lipodystrophy; laminopathies; lamins; mandibuloacral dysplasia; nuclear envelope proteins