Table 3.

Effect of compounds on cell survival.

			Survival (%) ± SEM
		μM	Mut MEFs	WT astrocytes	Mut astrocytes
Sig1R binders	pre084	0.1	100 ± 2.1
		1	101 ± 3.5	114 ± 7.9	97 ± 0.5
		10	99 ± 1.5	95 ± 2.3	93 ± 5.1
		20	97 ± 2.1
		30	105 ± 1.9	82 ± 1.9 (p = 8.3E–04)	76 ± 4.9 (p = 0.03)
		50	80 ± 2.8 (p = 0.01)
		100	57 ± 2.2 (p = 1.5E–05)
	Pridopidine	0.1	102 ± 1.7
		1	103 ± 1.3	103 ± 3.5	97 ± 2.7
		10	98 ± 1.6	101 ± 2.2	92 ± 2.8
		20	99 ± 0.64
		30	99 ± 3.1	85 ± 4.3 (p = 0.02)	97 ± 5.0
		50	97 ± 1.2
		100	91 ± 2.4 (p = 0.03)
	∗H8	0.1	104 ± 4.6
		1	102 ± 3.3	132 ± 10.7	108 ± 0.6
		10	99 ± 2.6	102 ± 3.8	93 ± 2.1
		20
		30	105 ± 13	99 ± 3.2	92 ± 1.6 (p = 0.14)
		50
		100
	NE100	0.1	99 ± 2.1	98 ± 1.8
		1	98 ± 2.2	86 ± 1.1 (p = 1.5E–5)	94 ± 4.7
		10	88 ± 3.5 (p = 0.017)	79 ± 2.2 (p = 1.6E–4)	82 ± 4.3 (p = 0.01)
		30			65 ± 2.2 (p = 6.0E–06)
H8 analogs	H8-1	10	104 ± 2.7
		20	103 ± 4.7
		30	97 ± 3.9
	H8-2	10	92 ± 4.2
		20	94 ± 8.8
		30	95 ± 13.1
	H8-3	10	100 ± 7.2
		20	96 ± 4.1
		30	88 ± 4.5 (p = 0.09)
	H8-4	10	92 ± 8.5
		20	95 ± 4.2
		30	98 ± 5.4
	H8-5	10	92 ± 1.7
		20	100 ± 4.4
		30	98 ± 13.8
	H8-6	10	97 ± 5.1
		20	92 ± 1.1
		30	93 ± 5.2
	H8-7	10	98 ± 5.1
		20	91 ± 4.7
		30	88 ± 9.7 (p = 0.44)
	H8-8	10	101 ± 5.6
		20	99 ± 11
		30	90 ± 3.5
Hedgehog modulators	SAG	0.01	98 ± 2.1
		0.05	92 ± 3.2
		0.1	95 ± 2.1
		1	68 ± 2.7 (p = 1.9E–05)
	Cyclopamine	0.1	92 ± 2.5
		1	95 ± 4.1
		10	79 ± 1.4 (p = 0.02)

Primary MEFs and astrocytes isolated from Eif2b^R132H/R132H (Mut) or WT mice were incubated for 24 h with the indicated compounds/concentrations followed by Crystal Violet staining. Cell survival of untreated cells was set to 100%. Shown is % cell survival ± SEM of at least three repeats. Toxicity refers to survival rate below 90% (marked gray, p-values indicated).