Fig. 6

Unbiased proteomics confirms mitochondrial protein deficit in C9ALS iPSCs. Gene ontology (a) and STRING analysis (b) for the 806 proteins reduced ≥10% in C9ALS iPSCs, demonstrating enrichment for components of oxidative phosphorylation, mitochondrial ribosome, and RNA degradation pathways. Higher magnification views of boxed clusters in b are shown in c−e. Gene ontology (f) and STRING analysis (g) for the 961 proteins increased ≥10% in C9ALS iPSCs, showing enrichment for components of the proteasome, amino acid biosynthesis, and RNA transport pathways. Higher magnification views of boxed clusters in g are shown in h−j. FDR false discovery rate. All experiments performed in triplicate with two lines/condition