Human Molecular Genetics, 1999, Vol. 8, No. 10 1893-1900
© 1999 Oxford University Press
Review |
Nonsense-mediated mRNA decayin health and disease
Howard Hughes Medical Institute and Institute for GeneticMedicine, Johns Hopkins University School of Medicine, Baltimore,MD, USA
ABSTRACT
All eukaryotes possess the ability to detect and degradetranscripts harboring premature signals for the termination of translation.Despite the ubiquitous nature of nonsense-mediated mRNA decay (NMD)and its demonstrated role in the modulation of phenotypes resultingfrom selected nonsense alleles, very little is known regarding itsbasic mechanism or the selective pressure for complete evolutionaryconservation of this function. This review will present the currentmodels of NMD that have been generated during the study of model organismsand mammalian cells. The physiological burden of nonsense transcriptsand the emerging view that NMD plays a broad and critical role inthe regulation of gene expression will also be discussed. Such issuesare relevant to the proposal that pharmacological manipulation ofNMD will find therapeutic application.
FOOTNOTES
a To whom correspondenceshould be addressed. Tel: +1 410 614 0701; Fax: +1410 614 2256; Email: hdietz{at}welchlink.welch.jhu.edu
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