title: Altered nuclear functions in progeroid syndromes: a paradigm for aging research.
abstract: Syndromes of accelerated aging could provide an entry point for identifying and dissecting the cellular pathways that are involved in the development of age-related pathologies in the general population. However, their usefulness for aging research has been controversial, as it has been argued that these diseases do not faithfully reflect the process of natural aging. Here we review recent findings on the molecular basis of two progeroid diseases, Werner syndrome (WS) and Hutchinson-Gilford progeria syndrome (HGPS), and highlight functional connections to cellular processes that may contribute to normal aging.
authors.affiliation: Department of Molecular Microbiology and Immunology, Institute for Genetic Medicine, Keck School of Medicine, University of Southern California, Los Angeles, USA.
authors.forename: Baomin
authors.initials: B
authors.lastname: Li
authors.forename: Sonali
authors.initials: S
authors.lastname: Jog
authors.forename: Jose
authors.initials: J
authors.lastname: Candelario
authors.forename: Sita
authors.initials: S
authors.lastname: Reddy
authors.forename: Lucio
authors.initials: L
authors.lastname: Comai
mesh_terms: D000375:Aging / Q000378:metabolism*; D035843:Biomedical Research; D002843:Chromatin / Q000378:metabolism / Q000502:physiology; D005786:Gene Expression Regulation / Q000502:physiology; D006801:Humans; D034904:Lamin Type A; D009687:Nuclear Proteins / Q000378:metabolism / Q000502:physiology; D009698:Nucleoproteins / Q000378:metabolism* / Q000502:physiology; D011371:Progeria / Q000378:metabolism*; D011498:Protein Precursors / Q000378:metabolism / Q000502:physiology; D016615:Telomere / Q000378:metabolism* / Q000502:physiology; D014898:Werner Syndrome / Q000378:metabolism*
chemical_list: D002843:Chromatin; D034904:Lamin Type A; D009687:Nuclear Proteins; D009698:Nucleoproteins; D011498:Protein Precursors; C090764:prelamin A