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Connecting the dots: RNP granule network in health and disease

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ELSEVIER
DOI: 10.1016/j.bbamcr.2021.119058

关键词

RNP granule; ALS; Nuclear body; LLPS; MLO; Stress granule; Paraspeckle

资金

  1. Motor Neurone Disease Association Senior Non-Clinical Fellowship [Shelkovnikova/Oct17/968799]
  2. Academy of Medical Sciences Springboard award

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All cells contain membraneless RNP granules consisting of RNA and proteins, which play crucial roles in organizing cellular processes. The dynamic nature of RNP granules allows cells to adapt to different functional states and environments. Disruption of the RNP granule network may contribute to diseases such as ALS.
All cells contain ribonucleoprotein (RNP) granules - large membraneless structures composed of RNA and proteins. Recent breakthroughs in RNP granule research have brought a new appreciation of their crucial role in organising virtually all cellular processes. Cells widely exploit the flexible, dynamic nature of RNP granules to adapt to a variety of functional states and the ever-changing environment. Constant exchange of molecules between the different RNP granules connects them into a network. This network controls basal cellular activities and is remodelled to enable efficient stress response. Alterations in RNP granule structure and regulation have been found to lead to fatal human diseases. The interconnectedness of RNP granules suggests that the RNP granule network as a whole becomes affected in disease states such as a representative neurodegenerative disease amyotrophic lateral sclerosis (ALS). In this review, we summarize available evidence on the communication between different RNP granules and on the RNP granule network disruption as a primary ALS pathomechanism.

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