Table 1. Effects of strongest GMR-hFUS^R521C and GMR-hTDP-43^M337V suppressors on GMR-c9orf72(G4C2)₃₀-related photoreceptor neurodegeneration.

Column A	Column B	Column C	Column D	Column E	Column F	Column G
Exelixis insert	Fus modification	TDP-43 Modification	C9orf72 Modification	% c9orf72 Degeneration	% c9orf72 Degeneration	% c9orf72 Degeneration
				Week 1	Week 3	Week 6
c00800	S2	S3	S3	0	0	1.7
c01016	S2	S2	S3	2.6	12.2	58.5
c01295	S1	S1	S3	6.8	17.6	53.4
c01399	S2	S2	S1	14.2	18.4	50.5
c02909	S1	S2	S3	4.2	10.4	42.4
c03244	S2	S1	S3	8.1	12.9	34.9
c04205	S2	S1	S3	9	14.5	31.8
c04522	S1	S1	S3	0	8.9	46.5
c04582	S2	S2	S2	3.1	26.1	Not done
c05668	S2	S2	S3	1.5	3.1	18.2
c06325	S2	S1	S1	9.4	25.3	64.8
c07023	S2	S3	S3	4.8	9.8	46.1
d00123	S2	S2	S3	0	4.8	0
d00147	S2	S1	S1	4.4	26.1	Not done
d01275	S3	S3	S3	0.7	0.8	0.8
d01345	S2	S2	S3	1.9	9.6	36
d02380	S2	S1	S3	0	3.8	39.6
d02712	S1	S2	S2	7.4	8	70
d02769	S2	S2	S3	0	0	8.3
d02833	S1	S2	S2	0	7.1	Not done
d02874	S1	S1	S1	5.7	45.7	67.6
d03208	S2	S2	S3	0	8.7	6.1
d03562	S3	S3	S3	0.7	7	25.6
d04116	S1	S1	S3	10.8	24	46.2
d04883	S2	S2	S3	5.8	29.8	61.4
d05297	S2	S1	S1	6.6	26.9	Not done
d05884	S2	S1	S2	2.4	28.6	70.3
d06126	S3	S3	S2	8.9	53.4	Not done
d07098	S1	S1	S2	0	16.4	23.4
d08551	S2	S2	S3	0	0	0
d09937	S2	S2	S2	34.5	36.9	62.5
d10223	S2	S3	S2	0	18.4	60
d10376	S3	S2	S3	0	1.2	18.5
d10800	S2	S2	S3	0	0	0
d11666	S2	S2	S3	5.6	9.4	26.5
e00530	S2	S2	S3	0.7	3.3	16.5
e00651	S2	S1	S3	7.3	11.9	44.2
e00985	S2	S2	S3	11.1	20.6	51.6
e01238	S2	S2	S3	1.1	7.1	4
e01976	S2	S2	S3	0	5.9	12.5
e02098	S2	S2	S3	3.4	15.2	43.8
e02166	S2	S2	S1	6.9	37.9	55.2
e02234	S1	S2	S1	15.2	27.7	54.3
e02236	S2	S1	S1	8.2	34.2	59.6
e02284	S3	S3	S3	7	19.1	43.9
e02535	S2	S2	S3	13.5	19.4	67.6
e02684	S2	S1	S2	13.5	30.8	53.9
e02699	S3	S1	S3	7.1	10	29.2
e03409	S2	S2	S1	16.9	20.4	Not done
e04015	S2	S2	S2	0	11.9	27.7
e04545	S2	S2	S3	6.1	13.2	40.2
f01587	S1	S1	S2	10.1	16.5	12.8
f03242	S3	S2	S3	0	0	16.2
f03756	S2	S2	S3	1.3	13	11.1
f05408	S2	S3	S3	0	0	0
f06593	S3	S3	S3	0	0	0
c03600	S2	S2	NE	12.3	26.9	60.9
c04473	S2	S2	NE	18	26.5	65.9
c04797	S2	S2	NE	24.4	39.3	72.9
c05977	S2	S1	NE	9.2	29.4	Not done
c06293	S2	S2	NE	18.2	34.5	68.5
c06703	S1	S2	NE	32.3	61.1	82.4
c06744	S2	S2	NE	20.1	34.8	63.7
c07042	S1	S1	NE	18.9	40.9	73.3
c07155	S2	S2	NE	22.8	49.4	67.7
d04075	S2	S3	NE	12.4	26.7	Not done
d07488	S2	S2	NE	35.7	30.8	76.9
d08578	S2	S2	NE	26.5	51.5	77.4
d09179	S2	S1	NE	Not done	56.1	72.2
e00355	S2	S2	NE	22.5	31.7	60.3
e00777	S2	S2	NE	15.5	24.9	41.7
e00785	S2	S2	NE	24	32.9	75.6
e02741	S2	S2	NE	27.1	47.5	79
f02738	S1	S1	NE	18.3	22.4	76.8
c03467	S2	S2	E2	41.5	52.5	Not done
c03635	S2	S2	E3	23.6	73.1	95.7
c05849	S2	S1	E1	26.7	57.5	Not done
d02986	S2	S2	E3	13.3	60.2	82.3
d06616	S2	S2	E3	60	80	87.5
e02580	S2	S3	E3	28.4	59	91.5
e04200	S2	S2	E2	66.9	85	Not done
f01201	S2	S2	E3	58.3	78.3	98.2
f03408	S1	S1	E1	20.4	50	77.8
f05963	S1	S2	E3	12.5	68.3	80.8

Effects of 84 overlapping suppressors of GMR-hFUS^R521C and GMR-hTDP-43^M337V degenerative eye phenotypes in a GMR-c9orf72(G4C2)₃₀ model of progressive neurodegeneration. Insert number is listed in Column A, followed by the observed modification in the GMR-hFUS^R521C (Column B) and GMR-hTDP-43^M337V (Column C) screening strains. In Columns B and C, S1, S2, and S3 refer to the qualitative strength of suppression, where S1 is weak, S2 is moderate, and S3 is strong suppression. Column D depicts modification effect of the insertion on the GMR-c9orf72(G4C2)₃₀ model, where S is suppression, E is enhancement, and NE is no effect. Columns E, F, and G provide the penetrance of the neurodegenerative phenotype at weeks 1, 3, and 6. S1/E1 is suppression/enhancement at one time point, S2/E2 is suppression/enhancement at two time points, and S3/E3 is suppression/enhancement at all three time points. Crosses were performed in batches and each insert was compared to control crosses (GMR-c9orf72(G4C2)₃₀ crossed to isoA) for that particular batch (data not shown).