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Open AccessArticle

TDP-43 Regulation of AChE Expression Can Mediate ALS-Like Phenotype in Zebrafish

Team “Translational Research for Neuronlogical Diseases”, Institut Imagine Inserm U1163, Université de Paris; Sorbonne Université, Université Pierre et Marie Curie (UPMC), Université de Paris 06, Unité Mixte 75, Institut National de la Santé et de la Recherche Médicale (INSERM) Unité 1127, Centre National de la Recherche Scientifique (CNRS) Unité Mixte de Recherche 7225 Institut du Cerveau et de la Moelle Épinière (ICM), 75013 Paris, France
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Cells 2021, 10(2), 221; https://doi.org/10.3390/cells10020221
Received: 15 October 2020 / Revised: 13 January 2021 / Accepted: 13 January 2021 / Published: 22 January 2021
The “distal axonopathy” hypothesis in amyotrophic lateral sclerosis (ALS) proposes that pathological changes occur at the neuromuscular junction (NMJ) early in the disease. While acetylcholinesterase (AChE) plays an important role in the functionality of the NMJ, its potential role in ALS remains unexplored. Here, we identified AChE as a limiting factor regulating muscle/motor neuron connection in a vertebrate model of ALS. Knockdown of the TAR DNA-binding protein 43 (TDP-43) orthologue in zebrafish resulted in early defects of motor functions coupled with NMJ disassembly. We found that a partially depleted tdp-43 caused a decrease of ache expression. Importantly, human AChE overexpression reduced the phenotypic defects in the tdp-43 loss of function model, with amelioration of post- and pre-synaptic deficits at the NMJ. In conclusion, our results provide a better understanding of the role of TDP-43 in the NMJ organization and indicate AChE as a contributing factor in the pathology of ALS. In particular, it may be implicated in the early defects that characterize NMJs in this major neurodegenerative disorder. View Full-Text
Keywords: amyotrophic lateral sclerosis (ALS); TAR DNA-binding protein 43 (TDP-43); neuromuscular junction (NMJ); knockdown (KD); acetylcholinesterase (AChE) amyotrophic lateral sclerosis (ALS); TAR DNA-binding protein 43 (TDP-43); neuromuscular junction (NMJ); knockdown (KD); acetylcholinesterase (AChE)
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MDPI and ACS Style

Campanari, M.-L.; Marian, A.; Ciura, S.; Kabashi, E. TDP-43 Regulation of AChE Expression Can Mediate ALS-Like Phenotype in Zebrafish. Cells 2021, 10, 221. https://doi.org/10.3390/cells10020221

AMA Style

Campanari M-L, Marian A, Ciura S, Kabashi E. TDP-43 Regulation of AChE Expression Can Mediate ALS-Like Phenotype in Zebrafish. Cells. 2021; 10(2):221. https://doi.org/10.3390/cells10020221

Chicago/Turabian Style

Campanari, Maria-Letizia; Marian, Anca; Ciura, Sorana; Kabashi, Edor. 2021. "TDP-43 Regulation of AChE Expression Can Mediate ALS-Like Phenotype in Zebrafish" Cells 10, no. 2: 221. https://doi.org/10.3390/cells10020221

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