期刊
HAEMATOLOGICA-THE HEMATOLOGY JOURNAL
卷 96, 期 6, 页码 905-913出版社
FERRATA STORTI FOUNDATION
DOI: 10.3324/haematol.2010.039206
关键词
mRNA stability; mRNA quality control; nonsense-mediated mRNA decay (NMD); beta-globin; beta-thalassemia; clinical phenotype
类别
资金
- Fundacao Luso-Americana para o Desenvolvimento
- Center for Biodiversity, Functional and Integrative Genomics from Fundacao para a Ciencia e a Tecnologia
- Fundacao para a Ciencia e a Tecnologia
Messenger RNA (mRNA) stability is a critical determinant that affects gene expression. Many pathways have evolved to modulate mRNA stability in response to developmental, physiological and/or environmental stimuli. Eukaryotic mRNAs have a considerable range of half-lives, from as short as a few minutes to as long as several days. Human globin mRNAs constitute an example of highly stable mRNAs. However, a wide variety of naturally occurring mutations that result in the clinical syndrome of thalassemia can trigger accelerated mRNA decay thus controlling mRNA quality prior to translation. Distinct surveillance mechanisms have been described as being targeted for specific defective globin mRNAs. Here, we review mRNA stability mechanisms implicated in the control of beta-globin gene expression and the surveillance pathways that prevent translation of aberrant beta-globin mRNAs. In addition, we emphasize the importance of these pathways in modulating the severity of the beta-thalassemia phenotype.
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