The Nucleolus: A Multiphase Condensate Balancing Ribosome Synthesis and Translational Capacity in Health, Aging and Ribosomopathies
The nucleolus is the largest membrane-less structure in the eukaryotic nucleus. It is involved in the biogenesis of ribosomes, essential macromolecular machines responsible for synthesizing all proteins required by the cell. The assembly of ribosomes is evolutionarily conserved and is the most energ...
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| Format: | Article |
| Language: | English |
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MDPI AG
2019-08-01
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| Series: | Cells |
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| Online Access: | https://www.mdpi.com/2073-4409/8/8/869 |
| _version_ | 1797715138256568320 |
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| author | Carl C. Correll Jiri Bartek Miroslav Dundr |
| author_facet | Carl C. Correll Jiri Bartek Miroslav Dundr |
| author_sort | Carl C. Correll |
| collection | DOAJ |
| description | The nucleolus is the largest membrane-less structure in the eukaryotic nucleus. It is involved in the biogenesis of ribosomes, essential macromolecular machines responsible for synthesizing all proteins required by the cell. The assembly of ribosomes is evolutionarily conserved and is the most energy-consuming cellular process needed for cell growth, proliferation, and homeostasis. Despite the significance of this process, the intricate pathophysiological relationship between the nucleolus and protein synthesis has only recently begun to emerge. Here, we provide perspective on new principles governing nucleolar formation and the resulting multiphase organization driven by liquid-liquid phase separation. With recent advances in the structural analysis of ribosome formation, we highlight the current understanding of the step-wise assembly of pre-ribosomal subunits and the quality control required for proper function. Finally, we address how aging affects ribosome genesis and how genetic defects in ribosome formation cause ribosomopathies, complex diseases with a predisposition to cancer. |
| first_indexed | 2024-03-12T08:02:27Z |
| format | Article |
| id | doaj.art-f1be3185dad94048aa89e8e0083ad9e4 |
| institution | Directory Open Access Journal |
| issn | 2073-4409 |
| language | English |
| last_indexed | 2024-03-12T08:02:27Z |
| publishDate | 2019-08-01 |
| publisher | MDPI AG |
| record_format | Article |
| series | Cells |
| spelling | doaj.art-f1be3185dad94048aa89e8e0083ad9e42023-09-02T19:46:55ZengMDPI AGCells2073-44092019-08-018886910.3390/cells8080869cells8080869The Nucleolus: A Multiphase Condensate Balancing Ribosome Synthesis and Translational Capacity in Health, Aging and RibosomopathiesCarl C. Correll0Jiri Bartek1Miroslav Dundr2Center for Proteomics and Molecular Therapeutics, Rosalind Franklin University of Medicine & Science, North Chicago, IL 60064, USADanish Cancer Society Research Center, Genome Integrity Unit, DK-2100 Copenhagen, DenmarkCenter for Cancer Cell Biology Immunology and Infection, Rosalind Franklin University of Medicine & Science, North Chicago, IL 60064, USAThe nucleolus is the largest membrane-less structure in the eukaryotic nucleus. It is involved in the biogenesis of ribosomes, essential macromolecular machines responsible for synthesizing all proteins required by the cell. The assembly of ribosomes is evolutionarily conserved and is the most energy-consuming cellular process needed for cell growth, proliferation, and homeostasis. Despite the significance of this process, the intricate pathophysiological relationship between the nucleolus and protein synthesis has only recently begun to emerge. Here, we provide perspective on new principles governing nucleolar formation and the resulting multiphase organization driven by liquid-liquid phase separation. With recent advances in the structural analysis of ribosome formation, we highlight the current understanding of the step-wise assembly of pre-ribosomal subunits and the quality control required for proper function. Finally, we address how aging affects ribosome genesis and how genetic defects in ribosome formation cause ribosomopathies, complex diseases with a predisposition to cancer.https://www.mdpi.com/2073-4409/8/8/869genome instabilitynucleolusphase separationpre-rRNA processingrDNA genesribosome biogenesisribosomal geneslinks to diseases |
| spellingShingle | Carl C. Correll Jiri Bartek Miroslav Dundr The Nucleolus: A Multiphase Condensate Balancing Ribosome Synthesis and Translational Capacity in Health, Aging and Ribosomopathies Cells genome instability nucleolus phase separation pre-rRNA processing rDNA genes ribosome biogenesis ribosomal genes links to diseases |
| title | The Nucleolus: A Multiphase Condensate Balancing Ribosome Synthesis and Translational Capacity in Health, Aging and Ribosomopathies |
| title_full | The Nucleolus: A Multiphase Condensate Balancing Ribosome Synthesis and Translational Capacity in Health, Aging and Ribosomopathies |
| title_fullStr | The Nucleolus: A Multiphase Condensate Balancing Ribosome Synthesis and Translational Capacity in Health, Aging and Ribosomopathies |
| title_full_unstemmed | The Nucleolus: A Multiphase Condensate Balancing Ribosome Synthesis and Translational Capacity in Health, Aging and Ribosomopathies |
| title_short | The Nucleolus: A Multiphase Condensate Balancing Ribosome Synthesis and Translational Capacity in Health, Aging and Ribosomopathies |
| title_sort | nucleolus a multiphase condensate balancing ribosome synthesis and translational capacity in health aging and ribosomopathies |
| topic | genome instability nucleolus phase separation pre-rRNA processing rDNA genes ribosome biogenesis ribosomal genes links to diseases |
| url | https://www.mdpi.com/2073-4409/8/8/869 |
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