Table 1.

Aberrant miRNAs in ALS.

MiRNA		Model	Function and Changes	Ref
Animal model	miRNA-125b	SOD1-G93A mouse	Increase of TNFα transcription by interfering with the STAT3 pathway	[44]
	miRNA-124		Aberrant modulation of astrocytic differentiation by targeting Sox2 and Sox9	[45]
	miRNA-128		Aberrant modulation of the excitatory amino acid transporter 2	[46]
	miRNA-193b-3p		Stimulation of cell death by targeting tuberous sclerosis 1 (TSC1)	[47]
	miRNA-375-3p	Wobbler mouse	Inefficient inhibition of p53, increasing ROS production, and induction of cell death	[48]
ALS patients	miRNA-146*	sALS	Interaction with NFL mRNA, and suppression of its expression	[49]
	miRNA-524-5p miRNA-582-3p	sALS	Aberrant regulation of NFL mRNA 3′UTR	[49]
	miRNA-375	FUS mutant ALS	Aberrant targeting of the apoptotic factor ELAV-like protein 4	[50]
	miRNA-494-3p	C9orf72 mutant ALS	Aberrant regulation of semaphorin 3A	[51]
	miRNA-223-3p miRNA-338-39 miRNA-326	sALS	Altered homeostasis in the neuromuscular junction	[52]
	miRNA-1 miRNA-19a-3p miRNA-133a-3p miRNA-133b miRNA-144-5p miRNA-192-3p miRNA-192-5p	fALS and sALS	Altered homeostasis in the serum	[53]
	Let-7d3p miRNA-139-5p miRNA-320a miRNA-320c miRNA-320b miRNA-425-5p	sALS and fALS	Altered homeostasis in the serum	[53]
	miRNA-1825	sALS and fALS	Overtranslation of tubulin-folding cofactor b, which contributes to the degradation of tubulin alpha-4A	[54]
	miRNA-424 miRNA-206	ALS	Altered homeostasis in the plasma and in the skeletal muscle	[55]
	miRNA-338-3p	sALS	Altered homeostasis in leukocytes	[56]
	miRNA-206	sALS	Altered homeostasis in the serum (no significant change in the serum over time)	[57]
	miRNA-143-3p miRNA-374b-5p	sALS	Altered homeostasis in the serum (significant change in the serum over time)	[57]
	miRNA-181a-5p miRNA-21-5p miRNA-15b-5p	ALS	Alteration of apoptotic mechanism inhibition and cell proliferation	[40]