--- res: bibo_abstract: - Premature aging disorders provide an opportunity to study the mechanisms that drive aging. In Hutchinson-Gilford progeria syndrome (HGPS), a mutant form of the nuclear scaffold protein lamin A distorts nuclei and sequesters nuclear proteins. We sought to investigate protein homeostasis in this disease. Here, we report a widespread increase in protein turnover in HGPS-derived cells compared to normal cells. We determine that global protein synthesis is elevated as a consequence of activated nucleoli and enhanced ribosome biogenesis in HGPS-derived fibroblasts. Depleting normal lamin A or inducing mutant lamin A expression are each sufficient to drive nucleolar expansion. We further show that nucleolar size correlates with donor age in primary fibroblasts derived from healthy individuals and that ribosomal RNA production increases with age, indicating that nucleolar size and activity can serve as aging biomarkers. While limiting ribosome biogenesis extends lifespan in several systems, we show that increased ribosome biogenesis and activity are a hallmark of premature aging.@eng bibo_authorlist: - foaf_Person: foaf_givenName: Abigail foaf_name: Buchwalter, Abigail foaf_surname: Buchwalter - foaf_Person: foaf_givenName: Martin W foaf_name: HETZER, Martin W foaf_surname: HETZER foaf_workInfoHomepage: http://www.librecat.org/personId=86c0d31b-b4eb-11ec-ac5a-eae7b2e135ed orcid: 0000-0002-2111-992X bibo_doi: 10.1038/s41467-017-00322-z bibo_volume: 8 dct_date: 2017^xs_gYear dct_isPartOf: - http://id.crossref.org/issn/2041-1723 dct_language: eng dct_publisher: Springer Nature@ dct_subject: - General Physics and Astronomy - General Biochemistry - Genetics and Molecular Biology - General Chemistry dct_title: Nucleolar expansion and elevated protein translation in premature aging@ fabio_hasPubmedId: '28855503' ...