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. 2021 Jul 14;12:713963. doi: 10.3389/fphar.2021.713963

TABLE 3.

Drugs identified for ALS treatment in zebrafish models.

Compound Classification Model Effects on phenotype Behavioral method Molecular mechanism References
FPL64176 and Bay K8644 L-type calcium channel agonists TARDBP-G348C-Tg Rescue impaired locomotor function Touch-evoked escape response, 52–56 hpf Rescue motoneuron excitability, decrease synaptic fidelity, NMJ abnormal structures Armstrong and Drapeau, (2013)
Pimozide T-type Ca2+ channels antagonist TARDBP-G348C-Tg; SOD1-G93A-Tg; FUS-R521H-Tg Improve motor defects, stabilize neuromuscular transmission Touch-evoked escape response, 52–56 hpf Not applicable Patten et al. (2017)
TRVA242 Pimozide derivatives TARDBP-G348C-Tg; SOD1- G93A-Tg; C9ORF72 Restores aberrant spinal motor neuron outgrowth; recovers NMJ structures and synaptic deficits Locomotion test, 5 dpf Not applicable Bose et al. (2019)
Riluzole Sodium current INaP inhibitor SOD1-G93R-Tg Reverse abnormal movements Touch-evoked escape response, 96 hpf; locomotion test, 12 months Reduce neuronal stress Benedetti et al. (2016)
Methylene blue Cationic thiazine dye TARDBP-G348C-Tg and FUS-R521H-Tg Protect impaired motor phenotypes Touch-evoked escape response, 48 hpf Inhibit TDP-43 aggregates, suppress oxidative stress Vaccaro et al. (2012)
1-Fe Corrole iron complex SOD1-G93R-Tg Improve motor defects Light-dark test, 6 dpf Anti-antioxidant Soll et al. (2021)
Ciprofloxacin and celecoxib Antibiotics and anti-inflammatory SOD1-G93R-Tg; TARDBP-G348C-Tg Rescue motor defects and axonopathy, recover NMJ structures and ramified morphology of microglia Touch-evoked escape response, 54–56 hpf; light-dark test, 6 dpf Anti-inflammatory Goldshtein et al. (2020)
Terbutaline sulfate β2-adrenergic receptor agonist TARDBP-Q331K-Tg Prevent axonal defects and NMJ degeneration Not applicable Activate β2-adrenergic receptors Paik et al. (2015)
PFI-1 Bromodomain inhibitor PR20 Improve embryos viability Not applicable Not applicable Corman et al. (2019)
Na-Phen Histone deacetylase inhibitor PR20 Improve embryos viability Not applicable Not applicable Corman et al. (2019)
Telbivudine Uracil-like nucleoside compound SOD1-W32S-Tg Rescue axonopathy and motor neuron deficits Touch-evoked escape response, 48 hpf Interact with W32 residue DuVal et al. (2019)