title: The nuclear lamina and its functions in the nucleus.
abstract: The nuclear lamina is a structure near the inner nuclear membrane and the peripheral chromatin. It is composed of lamins, which are also present in the nuclear interior, and lamin-associated proteins. The increasing number of proteins that interact with lamins and the compound interactions between these proteins and chromatin-associated proteins make the nuclear lamina a highly complex but also a very exciting structure. The nuclear lamina is an essential component of metazoan cells. It is involved in most nuclear activities including DNA replication, RNA transcription, nuclear and chromatin organization, cell cycle regulation, cell development and differentiation, nuclear migration, and apoptosis. Specific mutations in nuclear lamina genes cause a wide range of heritable human diseases. These diseases include Emery-Dreifuss muscular dystrophy, limb girdle muscular dystrophy, dilated cardiomyopathy (DCM) with conduction system disease, familial partial lipodystrophy (FPLD), autosomal recessive axonal neuropathy (Charcot-Marie-Tooth disorder type 2, CMT2), mandibuloacral dysplasia (MAD), Hutchison Gilford Progeria syndrome (HGS), Greenberg Skeletal Dysplasia, and Pelger-Huet anomaly (PHA). Genetic analyses in Caenorhabditis elegans, Drosophila, and mice show new insights into the functions of the nuclear lamina, and recent structural analyses have begun to unravel the molecular structure and assembly of lamins and their associated proteins.
authors.affiliation: Department of Genetics, The Institute of Life Sciences, The Hebrew University of Jerusalem, Jerusalem 91904, Israel.
authors.forename: Yosef
authors.initials: Y
authors.lastname: Gruenbaum
authors.forename: Robert D
authors.initials: RD
authors.lastname: Goldman
authors.forename: Ronit
authors.initials: R
authors.lastname: Meyuhas
authors.forename: Erez
authors.initials: E
authors.lastname: Mills
authors.forename: Ayelet
authors.initials: A
authors.lastname: Margalit
authors.forename: Alexandra
authors.initials: A
authors.lastname: Fridkin
authors.forename: Yaron
authors.initials: Y
authors.lastname: Dayani
authors.forename: Miron
authors.initials: M
authors.lastname: Prokocimer
authors.forename: Avital
authors.initials: A
authors.lastname: Enosh
mesh_terms: D000818:Animals; D002467:Cell Nucleus / Q000502:physiology* / Q000648:ultrastructure; D030342:Genetic Diseases, Inborn / Q000235:genetics / Q000503:physiopathology*; D006801:Humans; D034881:Nuclear Lamina / Q000502:physiology* / Q000648:ultrastructure; D009687:Nuclear Proteins / Q000502:physiology*
chemical_list: D009687:Nuclear Proteins