Research Paper Volume 14, Issue 24 pp 9832—9859

Figure 4. Expression patterns of ALS muscle circRNAs in iPSC-derived motor neurons bearing ALS-associated C9ORF72 mutations. (A) Representative images of day-32 motor neurons derived from iPSCs of a healthy subject (CS83iCTR – Table 3) and a C9-ALS patient (CS28iALS – Table 3). Motor neurons were stained for SMI32 and ISL1, nuclei were stained with DAPI. Scale bar indicates, 300 μm. (B, C) RT-qPCR analysis of iPSC-derived motor neurons from healthy controls (n = 4) and ALS patients bearing C9ORF72 mutations (n = 3) to measure the levels of circRNAs that were differentially upregulated (B) or downregulated (C) in human ALS muscle (Figure 2). Data were normalized to RPS9 mRNA levels, and TBP mRNA levels were included as controls; p-values ^*p < 0.05, ^**p < 0.01, ^***p < 0.001.