title: Lamins as mediators of oxidative stress.
abstract: The nuclear lamina defines both structural and functional properties of the eukaryotic cell nucleus. Mutations in the LMNA gene, encoding A-type lamins, lead to a broad spectrum of diseases termed laminopathies. While different hypotheses have been postulated to explain disease development, there is still no unified view on the mechanistic basis of laminopathies. Recent observations indicate that laminopathies are often accompanied by altered levels of reactive oxygen species and a higher susceptibility to oxidative stress at the cellular level. In this review, we highlight the role of reactive oxygen species for cell function and disease development in the context of laminopathies and present a framework of non-exclusive mechanisms to explain the reciprocal interactions between a dysfunctional lamina and altered redox homeostasis.
authors.affiliation: Cell Systems and Cellular Imaging Group, Department of Molecular Biotechnology, Ghent University, Ghent, Belgium.
authors.forename: Tom
authors.initials: T
authors.lastname: Sieprath
authors.forename: Rabih
authors.initials: R
authors.lastname: Darwiche
authors.forename: Winnok H
authors.initials: WH
authors.lastname: De Vos
mesh_terms: D006801:Humans; D034904:Lamin Type A / Q000235:genetics / Q000378:metabolism; D034882:Lamins / Q000235:genetics / Q000378:metabolism*; D008060:Lipodystrophy / Q000235:genetics; D009136:Muscular Dystrophies / Q000235:genetics; D034881:Nuclear Lamina / Q000378:metabolism*; D018384:Oxidative Stress; D011371:Progeria / Q000235:genetics; D017382:Reactive Oxygen Species / Q000378:metabolism*
chemical_list: C494847:LMNA protein, human; D034904:Lamin Type A; D034882:Lamins; D017382:Reactive Oxygen Species