Fig. 6

Downregulated miR-18b (miR-18b-5p) by SOD1 mutations contributes apoptotic cell death in SOD1(G93A) Tg mice and fALS patient spinal cord tissues. a Hif1α, Mef2c and Bax expression were increased in G93A mice. Mctp1, Rarb and Bcl2 proteins were decreased in G93A mice. b mRNAs of Hif1α, Mef2c and Bax was highly expressed in G93A mice. Mctp1, Rarb and Bcl2 transcripts were significantly downregulated in G93A mice. c miR-18b (miR-18b-5p) was deeply reduced and miR-206 was dramatically induced in G93A mice (n = 5). d The protein levels of Hif1α, Mef2c and Bax was upregulated in fALS (G86S) patient (Cervical and lumber). Mctp1, Rarb and Bcl2 proteins were decreased in fALS (G86S) patient. Normal spinal cord tissues (Cervical (control 1 and 2) served as a negative control (Cont). e The transcripts of Hif1α, Mef2c and Bax was highly upregulated in fALS (G86S) patient (Cervicals). Mctp1, Rarb and Bcl2 transcripts were significantly downregulated in fALS (G86S) patient. f miR-18b (miR-18b-5p) expression was importantly decreased and miR-206 was highly expressed in fALS (G86S) patient (Cervial and Lumber). Normal spinal cord tissues (Cervicals (control 1 and 2) served as a negative control (Cont). hSOD1 immunoreactivity on western blots of the insoluble fraction of the G93A mice and fALS (G86S) patients tissues. Significantly different at *, p < 0.05; **, p < 0.005