Please use this identifier to cite or link to this item: https://doi.org/10.1155/2018/8413496
Title: Synaptic paths to neurodegeneration: The emerging role of TDP-43 and FUS in synaptic functions
Authors: Ling, S.-C 
Keywords: DNA binding protein
RNA binding protein FUS
TDP-43 protein, human
degenerative disease
human
metabolism
nerve degeneration
synapse
DNA-Binding Proteins
Humans
Nerve Degeneration
Neurodegenerative Diseases
RNA-Binding Protein FUS
Synapses
Issue Date: 2018
Citation: Ling, S.-C (2018). Synaptic paths to neurodegeneration: The emerging role of TDP-43 and FUS in synaptic functions. Neural Plasticity 2018 : 8413496. ScholarBank@NUS Repository. https://doi.org/10.1155/2018/8413496
Rights: Attribution 4.0 International
Abstract: TAR DNA-binding protein-43 KDa (TDP-43) and fused in sarcoma (FUS) as the defining pathological hallmarks for amyotrophic lateral sclerosis (ALS) and frontotemporal dementia (FTD), coupled with ALS-FTD-causing mutations in both genes, indicate that their dysfunctions damage the motor system and cognition. On the molecular level, TDP-43 and FUS participate in the biogenesis and metabolism of coding and noncoding RNAs as well as in the transport and translation of mRNAs as part of cytoplasmic mRNAribonucleoprotein (mRNP) granules. Intriguingly, many of the RNA targets of TDP-43 and FUS are involved in synaptic transmission and plasticity, indicating that synaptic dysfunction could be an early event contributing to motor and cognitive deficits in ALS and FTD. Furthermore, the ability of the low-complexity prion-like domains of TDP-43 and FUS to form liquid droplets suggests a potential mechanism for mRNP assembly and conversion. This review will discuss the role of TDP-43 and FUS in RNA metabolism, with an emphasis on the involvement of this process in synaptic function and neuroprotection. This will be followed by a discussion of the potential phase separation mechanism for forming RNP granules and pathological inclusions. © 2018 Shuo-Chien Ling.
Source Title: Neural Plasticity
URI: https://scholarbank.nus.edu.sg/handle/10635/182107
ISSN: 20905904
DOI: 10.1155/2018/8413496
Rights: Attribution 4.0 International
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