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Curr Opin Genet Dev. 2013 Feb;23(1):63-71. doi: 10.1016/j.gde.2013.02.001. Epub 2013 Mar 13.

Growth control and ribosomopathies.

Author information

1
Department of Internal Medicine, Division of Hematology/Oncology, College of Medicine, University of Cincinnati, 3125 Eden Avenue, Cincinnati, OH 45267, USA.

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.

PMID:
23490481
DOI:
10.1016/j.gde.2013.02.001
[Indexed for MEDLINE]

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