Abstract：
:Recent genetic and biochemical evidence has improved our understanding of the pathomechanisms that lead to amyotrophic lateral sclerosis (ALS) and frontotemporal dementia (FTD), two devastating neurodegenerative diseases with overlapping symptoms and causes. Impaired RNA metabolism, enhanced aggregation of protein-RNA complexes, aberrant formation of ribonucleoprotein (RNP) granules and dysfunctional protein clearance via autophagy are emerging as crucial events in ALS/FTD pathogenesis. Importantly, these processes interact at the molecular level, converging on a common pathogenic cascade. In this review, we summarize key principles underlying ALS and FTD, and we discuss how mutations in genes involved in RNA metabolism, protein quality control and protein degradation meet mechanistically to impair the functionality and dynamics of RNP granules, and how this leads to cellular toxicity and death. Finally, we describe recent advances in understanding signaling pathways that become dysfunctional in ALS/FTD, partly due to altered RNP granule dynamics, but also with stress granule-independent mechanisms and, thus could be promising targets for future therapeutic intervention.
journal_name
Semin Cell Dev Bioljournal_title
Seminars in cell & developmental biologyauthors
Mandrioli J,Mediani L,Alberti S,Carra Sdoi
10.1016/j.semcdb.2019.06.003subject
Has Abstractpub_date
2020-03-01 00:00:00pages
183-192eissn
1084-9521issn
1096-3634pii
S1084-9521(18)30200-3journal_volume
99pub_type
杂志文章,评审abstract：:During nervous system development stem cell daughters must exit the proliferative cycle to adopt specific neural and glial fates and they must do so in the correct positions. Cell proliferation in the central nervous system occurs in neuroepithelia such as the neural retina and the ventricular zones. As cells are assi...
journal_title：Seminars in cell & developmental biology
pub_type： 杂志文章,评审
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abstract：:Myocardial infarction results in scar tissue that cannot actively contribute to heart mechanical function and frequently causes lethal arrhythmias. The healing response after infarction involves inflammation, biochemical signaling, changes in cellular phenotype, activity, and organization, and alterations in electrica...
journal_title：Seminars in cell & developmental biology
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abstract：:Lipid droplets (LDs) are versatile organelles with central roles in lipid and energy metabolism in all eukaryotes. They primarily buffer excess fatty acids by storing them as neutral lipids, mainly triglycerides and steryl esters. The neutral lipids form a core, surrounded by a unique phospholipid monolayer coated wit...
journal_title：Seminars in cell & developmental biology
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journal_title：Seminars in cell & developmental biology
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journal_title：Seminars in cell & developmental biology
pub_type： 杂志文章,评审
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journal_title：Seminars in cell & developmental biology
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journal_title：Seminars in cell & developmental biology
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journal_title：Seminars in cell & developmental biology
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journal_title：Seminars in cell & developmental biology
pub_type： 杂志文章,评审
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journal_title：Seminars in cell & developmental biology
pub_type： 杂志文章,评审
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journal_title：Seminars in cell & developmental biology
pub_type： 杂志文章,评审
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journal_title：Seminars in cell & developmental biology
pub_type： 杂志文章,评审
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journal_title：Seminars in cell & developmental biology
pub_type： 杂志文章,评审
doi：10.1016/j.semcdb.2015.06.006
更新日期：2015-07-01 00:00:00
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journal_title：Seminars in cell & developmental biology
pub_type： 杂志文章,评审
doi：10.1016/j.semcdb.2015.10.014
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journal_title：Seminars in cell & developmental biology
pub_type： 杂志文章,评审
doi：10.1016/j.semcdb.2011.10.006
更新日期：2011-12-01 00:00:00
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journal_title：Seminars in cell & developmental biology
pub_type： 杂志文章,评审
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journal_title：Seminars in cell & developmental biology
pub_type： 杂志文章,评审
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journal_title：Seminars in cell & developmental biology
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journal_title：Seminars in cell & developmental biology
pub_type： 杂志文章,评审
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abstract：:The Publisher regrets that this article is an accidental duplication of an article that has already been published, http://dx.doi.org/10.1016/j.semcdb.2013.05.010. The duplicate article has therefore been withdrawn. ...
journal_title：Seminars in cell & developmental biology
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doi：10.1016/j.semcdb.2013.05.011
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journal_title：Seminars in cell & developmental biology
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journal_title：Seminars in cell & developmental biology
pub_type： 杂志文章,评审
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journal_title：Seminars in cell & developmental biology
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journal_title：Seminars in cell & developmental biology
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abstract：:Axonal projections between two populations of neurons, which preserve neighborhood relationships, are called topographic. They are ubiquitous in the brain. The development of the retinotectal projection, mapping the retinal output onto the roof of the midbrain, has been studied for decades as a model system. The rigid...
journal_title：Seminars in cell & developmental biology
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abstract：:Plant oils represent an energy-rich and carbon-dense group of hydrophobic compounds. These oils are not only of economic interest, but also play important, fundamental roles in plant and algal growth and development. The subcellular storage compartments of plant lipids, referred to as lipid droplets (LDs), have long b...
journal_title：Seminars in cell & developmental biology
pub_type： 杂志文章,评审
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journal_title：Seminars in cell & developmental biology
pub_type： 杂志文章,评审
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更新日期：2017-04-01 00:00:00
abstract：:The ancestors of modern Metazoa were constructed in large part by the foldings and distortions of two-dimensional sheets of epithelial cells. This changed approximately 600 million years ago with the evolution of mesenchymal cells. These cells arise as the result of epithelial cell delamination through a reprogramming...
journal_title：Seminars in cell & developmental biology
pub_type： 杂志文章,评审
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更新日期：2008-06-01 00:00:00
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journal_title：Seminars in cell & developmental biology
pub_type： 杂志文章,评审
doi：10.1016/j.semcdb.2018.09.006
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