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(1)

Uncropped western blots Figure 2A

hFUS No Tg

+/+ ∆/+ +/+ ∆/+ ∆/∆

Total FUS

Vinculin

75 kDa 50 kDa

150 kDa

C-ter FUS

Vinculin

75 kDa 50 kDa

150 kDa hFUS

No Tg

+/+ ∆/+ +/+ ∆/+ ∆/∆

(2)

Uncropped western blots Figure 2A (continued)

hFUS No Tg

+/+ ∆/+ +/+ ∆/+ ∆/∆

Mouse FUS

Vinculin

75 kDa 50 kDa

150 kDa

Human FUS

Vinculin

75 kDa 50 kDa

150 kDa hFUS

No Tg

+/+ ∆/+ +/+ ∆/+ ∆/∆

(3)

hFUS

Cytoplasm Nucleus No Tg

+/+ ∆/+ +/+ ∆/+ ∆/∆

hFUS No Tg

+/+ ∆/+ +/+ ∆/+ ∆/∆

N-ter FUS

C-ter FUS

mouse FUS

human FUS

hFUS

Cytoplasm Nucleus No Tg

+/+ ∆/+ +/+ ∆/+ ∆/∆

hFUS No Tg

+/+ ∆/+ +/+ ∆/+ ∆/∆

Uncropped western blots Figure 2C

75 kDa 50 kDa

75 kDa 50 kDa

75 kDa 50 kDa

75 kDa 50 kDa

(4)

HDAC1

SOD1

hFUS

Cytoplasm Nucleus No Tg

+/+ ∆/+ +/+ ∆/+ ∆/∆

hFUS No Tg

+/+ ∆/+ +/+ ∆/+ ∆/∆

hFUS

Cytoplasm Nucleus No Tg

+/+ ∆/+ +/+ ∆/+ ∆/∆

hFUS No Tg

+/+ ∆/+ +/+ ∆/+ ∆/∆

ADMA FUS

hFUS

Cytoplasm Nucleus No Tg

+/+ ∆/+ +/+ ∆/+ ∆/∆

hFUS No Tg

+/+ ∆/+ +/+ ∆/+ ∆/∆

hFUS

Cytoplasm Nucleus No Tg

+/+ ∆/+ +/+ ∆/+ ∆/∆

hFUS No Tg

+/+ ∆/+ +/+ ∆/+ ∆/∆

Uncropped western blots Figure 2C (continued)

75 kDa 50 kDa

25 kDa 20 kDa

75 kDa 50 kDa

(5)

Uncropped gels

Lumbar spinal cord 1 month

Lumbar spinal cord 22 months

Lumbar spinal cord 1 month

Lumbar spinal cord 22 months

Lumbar spinal cord 1 month

Lumbar spinal cord 22 months

Intron 6 retentionIntron 7 retentionIntron 7 skipping

Figure 5

500 bp 350 bp 200 bp

Intron 6 + (363 bp)

Intron 6 - (240 bp)

500 bp 350 bp 200 bp

Intron 6 + (363 bp)

Intron 6 - (240 bp)

500 bp 350 bp 200 bp

Intron 7 + (469 bp)

Intron 7 - (240 bp)

500 bp 350 bp 200 bp

Intron 7 + (469 bp)

Intron 7 - (240 bp)

500 bp 350 bp 200 bp

Exon 7 + (248 bp) Exon 7 - (213 bp)

500 bp 350 bp 200 bp

Exon 7 + (248 bp) Exon 7 - (213 bp)

(6)

Uncropped gels

Frontal cortex 1 month

Frontal cortex 22 months

Frontal cortex 1 month

Frontal cortex 22 months

Frontal cortex 1 month

Frontal cortex 22 months Figure S5

Intron 6 retentionIntron 7 retentionIntron 7 skipping

500 bp 350 bp 200 bp

Intron 6 + (363 bp)

Intron 6 - (240 bp)

500 bp 350 bp 200 bp

Intron 6 + (363 bp)

Intron 6 - (240 bp)

500 bp 350 bp 200 bp

Intron 7 + (469 bp)

Intron 7 - (240 bp)

500 bp 350 bp 200 bp

Intron 7 + (469 bp)

Intron 7 - (240 bp)

500 bp 350 bp 200 bp

Exon 7 + (248 bp)

Exon 7 - (213 bp)

500 bp 350 bp 200 bp

Exon 7 + (248 bp)

Exon 7 - (213 bp)

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