RT Journal Article
SR Electronic
T1 Cellular RNA surveillance in health and disease
JF Science
JO Science
FD American Association for the Advancement of Science
SP 822
OP 827
DO 10.1126/science.aax2957
VO 366
IS 6467
A1 Wolin, Sandra L.
A1 Maquat, Lynne E.
YR 2019
UL http://science.sciencemag.org/content/366/6467/822.abstract
AB The numerous quality control pathways that target defective ribonucleic acids (RNAs) for degradation play key roles in shaping mammalian transcriptomes and preventing disease. These pathways monitor most steps in the biogenesis of both noncoding RNAs (ncRNAs) and protein-coding messenger RNAs (mRNAs), degrading ncRNAs that fail to form functional complexes with one or more proteins and eliminating mRNAs that encode abnormal, potentially toxic proteins. Mutations in components of diverse RNA surveillance pathways manifest as disease. Some mutations are characterized by increased interferon production, suggesting that a major role of these pathways is to prevent aberrant cellular RNAs from being recognized as “non-self.” Other mutations are common in cancer, or result in developmental defects, revealing the importance of RNA surveillance to cell and organismal function.