Table 2.

Candidate drugs and compounds for ALS developed by iPSCs-disease models.

Name	Functions	Reference
Bosutinib	A Src/c-Abl inhibitor; increases the survival of ALS MNs, increases autophagy, reduces the amount of misfolded SOD1 protein.	[52]
Retigabine (ezogabine)	A Kv7 channel activator; blocks hyperexcitability, improves MN survival.	[80]
ROPI	A dopamine agonist; suppresses neurite retraction and cell death, inhibits oxidative stress, improves mitochondrial function, inhibits TDP-43 and FUS aggregation.	[111]
Compound #56	An inhibitor of SOD1-Derlin-1 interaction; ameliorates ALS pathology in MNs, delays onset and prolongs survival of ALS model mice.	[112]
Mitoxantrone	Contains extended planararomatic moieties; prevents TDP-43, FUS from forming SGs.	[113]
SAHA, RGFP109	Histone deacetylase inhibitors; reduce loss of nuclear FUS, rescue the DNA repair response (combined with arimoclomol).	[114]
Niclosamide	A STAT3 inhibitor; prevents TDP-43 mislocation, degrades TDP-43 aggregates, activates mitophagy, attenuates morphological changes.	[115]