Figure 1

The expression of DROSHA/DGCR8 is dysregulated in SMA motor neurons. (A) Western blots of DROSHA, DGCR8 and ACTB in 10DIV motor neurons (B) Quantification of Western blots, n = 12 (WT), n = 11 (SMA) for DROSHA, n = 4 (WT and SMA) for DGCR8. Each sample represents an individual embryo. (C) mRNA levels of Drosha and Dgcr8 were measured by qRT-PCR in 10DIV motor neurons: n = 20 (WT) and n = 12 (SMA) (D) Pie charts represent the composition of miRNAs in 10DIV motor neurons. miRNAs account for less than 1% of total reads were grouped as other miRs. Deep sequencing data show that total number of reads of miRNAs are reduced in SMA. (E) Bar graph representing qRT-PCR of primary miRNA transcripts: n = 14 (WT) and n = 13 (SMA) (F) Precursor miRNA levels: n = 15 (WT) and n = 18 (SMA) for miR-218-1 and miR-218-2, n = 10 (WT and SMA) for miR-183 (G) Mature miRNA levels: n = 34 (WT, except miR-10a-5p, miR-10b-5p and miR-218), n = 22 (SMA, except miR-10a-5p, miR-10b-5p and miR-218), n = 12 (WT, miR-218) and n = 10 (SMA, miR-218), n = 10 (WT and SMA, miR-10a-5p and miR-10b-5p) Data are represented as mean±SEM, Statistical significance is determined with t-test, *p < 0.05 and ***p < 0.001. ns = not significant.