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. Author manuscript; available in PMC: 2021 Nov 1.

Published in final edited form as: Adv Ther (Weinh). 2020 Aug 11;3(11):2000133. doi: 10.1002/adtp.202000133

Table 1.

Summary of the NMJ phenotype analysis for the three ALS lines and their response to DP treatment.

MN phenotype
ALS Lines	Viability	Axonal Branches		Axonal Length/Neuron		Dendritic Length/Neuron		Axonal varicosity	IK+ dr/INa+
		Day 1	Day 2	Day 1	Day 2	Day 1	Day 2		Wk 2	Wk 3	Wk 4
SOD1 L144P	^**	^***	^***	^***	^***	^***	^***	^***			^*
SOD1 D90A	^*	^**	^***	^***	^***	^***	^***	^***			^**
FUS G522A		^*	^**	^***	^***	^***	^*	^***
NMJ phenotype
ALS Lines	NMJ Number		NMJ Fidelity								Fatigue Index
	D14	D17	D14				D17				D 14		D17
			0.33 Hz	0.5 Hz	1 Hz	2 Hz	0.33 Hz	0.5 Hz	1 Hz	2 Hz	1 Hz	2 Hz	1 Hz	2 Hz
SOD1 L144P	^***				^*	^*			^*	^**	^***	^*	^***	^***
SOD1 D90A	^*			^*	^*	^**			^*	^*	^*	^*	^*
FUS G522A				^***	^**	^**		^*	^*	^*	^*	^**		^*
Treatment effect by DP
ALS Lines	NMJ Number		NMJ Fidelity								Fatigue Index
	D14	D17	D14				D17				Day 14		D17
			0.33 Hz	0.5 Hz	1 Hz	2 Hz	0.33 Hz	0.5 Hz	1 Hz	2 Hz	1 Hz	2 Hz	1 Hz	2 Hz
SOD1 L144P	^**	^*			^**	^*	^*	^*	^**	^**	^*		^***	^***
SOD1 D90A	^*	^*			^**	^***		^*	^*	^*		^*	^*
FUS G522A	^**	^**		^*	^*	^*					^*		^*	^*

^*

P < 0.1,

^**

P < 0.01,

^***

P < 0.001