title: Growth control and ribosomopathies.
abstract: Ribosome biogenesis and protein synthesis are two of the most energy consuming processes in a growing cell. Moreover, defects in their molecular components can alter the pattern of gene expression. Thus it is understandable that cells have developed a surveillance system to monitor the status of the translational machinery. Recent discoveries of causative mutations and deletions in genes linked to ribosome biogenesis have defined a group of similar pathologies termed ribosomopathies. Over the past decade, much has been learned regarding the relationship between growth control and ribosome biogenesis. The discovery of extra-ribosomal functions of several ribosome proteins and their regulation of p53 levels has provided a link from ribosome impairment to cell cycle regulation. Yet, evidence suggesting p53 and/or Hdm2 independent pathways also exists. In this review, we summarize recent advances in understanding the mechanisms underlying the pathologies of ribosomopathies and discuss the relationship between ribosome production and tumorigenesis.
authors.affiliation: Department of Internal Medicine, Division of Hematology/Oncology, College of Medicine, University of Cincinnati, 3125 Eden Avenue, Cincinnati, OH 45267, USA.
authors.forename: Teng
authors.initials: T
authors.lastname: Teng
authors.forename: George
authors.initials: G
authors.lastname: Thomas
authors.forename: Carol A
authors.initials: CA
authors.lastname: Mercer
mesh_terms: D000818:Animals; D002453:Cell Cycle; D006801:Humans; D009154:Mutation; D009369:Neoplasms / Q000235:genetics; D001678:Organelle Biogenesis; D014176:Protein Biosynthesis; D051736:Proto-Oncogene Proteins c-mdm2 / Q000235:genetics / Q000378:metabolism; D012335:RNA, Ribosomal / Q000096:biosynthesis / Q000235:genetics*; D012269:Ribosomal Proteins / Q000096:biosynthesis / Q000235:genetics; D012270:Ribosomes / Q000235:genetics* / Q000378:metabolism / Q000473:pathology*; D016159:Tumor Suppressor Protein p53 / Q000235:genetics / Q000378:metabolism
chemical_list: D012335:RNA, Ribosomal; D012269:Ribosomal Proteins; D016159:Tumor Suppressor Protein p53; C027624:ribosomal protein L11; C029742:ribosomal protein L5; D051736:Proto-Oncogene Proteins c-mdm2