Fig. 7: Smn1 deficient muscle cells accumulate autolysosomes and multivescicular bodies (MVBs). | Cell Death & Disease

Fig. 7: Smn1 deficient muscle cells accumulate autolysosomes and multivescicular bodies (MVBs).

From: Dysfunctional mitochondria accumulate in a skeletal muscle knockout model of Smn1, the causal gene of spinal muscular atrophy

Fig. 7

a Representative electron microscopy images showing increased number of autolysosomes and MVBs (white arrows) upon Smn1 knockdown in C2C12 myoblasts and differentiated myotubes (n = 3 independent experiments). Scale bar: 10 μm. b Representative high magnification of Smn1 deficient muscle cells. Scale bar: 500 nm. c Equal amounts of siScr and siSmn1 transfected C2C12 lysates (20 µg) were separated by SDS-PAGE and immunoblotted using the indicated antibodies. Where indicated the cells were treated with chloroquine (CQ) before collection. d Dots plot of the differences in the amount of LAMP2, LC3-II and p62 between samples in the presence and absence of CQ as a measure of the autophagic flux in siScr and siSmn1 transfected cells (n = 3 paired biological replicates represented by different colors). p value was calculated using ratio paired t test one tail statistical analysis; LAMP2 and LC3-II, *p = 0.04; p62, p = 0.07.

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