Neuromuscular junction abnormalities in a zebrafish loss-of-function model of TDP-43.
3-Pyridinecarboxylic acid, 1,4-dihydro-2,6-dimethyl-5-nitro-4-(2-(trifluoromethyl)phenyl)-, Methyl ester
/ pharmacology
Alleles
Animals
Animals, Genetically Modified
CRISPR-Cas Systems
Calcium Channel Agonists
/ pharmacology
Calcium Channels, L-Type
/ metabolism
DNA-Binding Proteins
/ genetics
Disease Models, Animal
Genotype
Loss of Function Mutation
Motor Activity
/ drug effects
Neuromuscular Junction
/ drug effects
Synaptic Transmission
/ drug effects
TDP-43 Proteinopathies
/ drug therapy
Zebrafish
Zebrafish Proteins
/ genetics
ALS
NMJ
TDP-43
zebrafish
Journal
Journal of neurophysiology
ISSN: 1522-1598
Titre abrégé: J Neurophysiol
Pays: United States
ID NLM: 0375404
Informations de publication
Date de publication:
01 01 2019
01 01 2019
Historique:
pubmed:
22
11
2018
medline:
23
8
2019
entrez:
22
11
2018
Statut:
ppublish
Résumé
Almost 90% of amyotrophic lateral sclerosis (ALS) cases are characterized by the presence of aggregates of insoluble, misfolded cytoplasmic TAR DNA binding protein of 43 kDa (TDP-43). Distal axonopathy with impaired neuromuscular junctions (NMJs) before motor neuron degeneration or clinical onset of symptoms has been hypothesized as an early pathology in ALS. However, synaptic defects at the NMJ caused by TDP-43 mutations have not been characterized. In this study, we examined a previously reported zebrafish line expressing the tardbp
Identifiants
pubmed: 30461368
doi: 10.1152/jn.00265.2018
doi:
Substances chimiques
Calcium Channel Agonists
0
Calcium Channels, L-Type
0
DNA-Binding Proteins
0
Tardbp protein, zebrafish
0
Zebrafish Proteins
0
3-Pyridinecarboxylic acid, 1,4-dihydro-2,6-dimethyl-5-nitro-4-(2-(trifluoromethyl)phenyl)-, Methyl ester
71145-03-4
Types de publication
Journal Article
Research Support, Non-U.S. Gov't
Langues
eng
Pagination
285-297Subventions
Organisme : CIHR
Pays : Canada