期刊
CELLS
卷 8, 期 3, 页码 -出版社
MDPI
DOI: 10.3390/cells8030229
关键词
ribosome; cancer; ribosomopathies; translational fidelity
类别
资金
- ERC starting grant [334946]
- FWO [G067015N]
- Stichting Tegen Kanker [2016-775, 2016-801]
- KU Leuven Research Council [C14/18/104]
- Jose Carreras EHA junior research grant
- Kom Op Tegen Kanker postdoctoral fellowship
- leukemia research grant from the Me To You Foundation
- European Research Council (ERC) [334946] Funding Source: European Research Council (ERC)
Ribosomopathies are congenital diseases with defects in ribosome assembly and are characterized by elevated cancer risks. Additionally, somatic mutations in ribosomal proteins have recently been linked to a variety of cancers. Despite a clear correlation between ribosome defects and cancer, the molecular mechanisms by which these defects promote tumorigenesis are unclear. In this review, we focus on the emerging mechanisms that link ribosomal defects in ribosomopathies to cancer progression. This includes functional onco-specialization of mutant ribosomes, extra-ribosomal consequences of mutations in ribosomal proteins and ribosome assembly factors, and effects of ribosomal mutations on cellular stress and metabolism. We integrate some of these recent findings in a single model that can partially explain the paradoxical transition from hypo- to hyperproliferation phenotypes, as observed in ribosomopathies. Finally, we discuss the current and potential strategies, and the associated challenges for therapeutic intervention in ribosome-mutant diseases.
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