title: Role of A-type lamins in signaling, transcription, and chromatin organization.
abstract: A-type lamins (lamins A and C), encoded by the LMNA gene, are major protein constituents of the mammalian nuclear lamina, a complex structure that acts as a scaffold for protein complexes that regulate nuclear structure and functions. Interest in these proteins has increased in recent years with the discovery that LMNA mutations cause a variety of human diseases termed laminopathies, including progeroid syndromes and disorders that primarily affect striated muscle, adipose, bone, and neuronal tissues. In this review, we discuss recent research supporting the concept that lamin A/C and associated nuclear envelope proteins regulate gene expression in health and disease through interplay with signal transduction pathways, transcription factors, and chromatin-associated proteins.
authors.affiliation: Department of Atherothrombosis and Cardiovascular Imaging, Fundación Centro Nacional de Investigaciones Cardiovasculares Carlos III, Madrid, Spain. vandres@cnic.es
authors.forename: Vicente
authors.initials: V
authors.lastname: Andrés
authors.forename: José M
authors.initials: JM
authors.lastname: González
mesh_terms: D002843:Chromatin / Q000378:metabolism*; D064211:Connectin; D005786:Gene Expression Regulation; D034904:Lamin Type A / Q000235:genetics / Q000378:metabolism / Q000502:physiology*; D008565:Membrane Proteins / Q000235:genetics / Q000378:metabolism / Q000502:physiology; D008954:Models, Biological; D024510:Muscle Development; D009124:Muscle Proteins / Q000378:metabolism; D009685:Nuclear Envelope / Q000378:metabolism; D011494:Protein Kinases / Q000378:metabolism; D051880:Receptors, Notch / Q000378:metabolism / Q000502:physiology; D015398:Signal Transduction; D014157:Transcription Factors / Q000235:genetics / Q000378:metabolism; D014158:Transcription, Genetic; D016212:Transforming Growth Factor beta / Q000378:metabolism / Q000502:physiology
chemical_list: D002843:Chromatin; D064211:Connectin; D034904:Lamin Type A; D008565:Membrane Proteins; D009124:Muscle Proteins; D051880:Receptors, Notch; C577285:TTN protein, human; D014157:Transcription Factors; D016212:Transforming Growth Factor beta; C440308:lamin C; D011494:Protein Kinases