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. Author manuscript; available in PMC: 2014 Jul 15.
Published in final edited form as: Neuron. 2013 Oct 16;80(2):415–428. doi: 10.1016/j.neuron.2013.10.015

Figure 3. C9RF72 ALS Cells Show Cytoplasmic RAN Translation Peptides.

Figure 3

(A) C9ORF72 iPSN cells contain cytoplasmic GGGGCC RNA foci, while control iPSNs do not.

(B) Quantification of cytoplasmic RNA foci reveals that cytoplasmic foci are RNase A sensitive.

(C) Single-optical plane (∼0.3 µm) image of C9ORF72 ALS motor cortex also shows cytoplasmic GGGGCC RNA foci similar to iPSNs.

(D) RAN protein shown by IF using C9RANT antibody that preferentially detects the poly-(Gly-Pro) RAN product in C9ORF72 iPSNs but not control iPSNs.

***p < 0.001. Scale bar = 5µm for (A), (D), and (E), right panels; 1 mm for (C), and 20 µm for (D) and (E), left panels. See also Figure S3B.