Fig. 9
From: A toxic gain-of-function mechanism in C9orf72 ALS impairs the autophagy-lysosome pathway in neurons
Phosphorylation of TBK1 at S172 is increased in C9orf72 MNs. a, Representative Western blot of iPSC-derived MNs immunostained for pTBK (S172), TBK1 and β-actin. b, c Quantifications of the Western blot shown in (a), measuring the relative phosphorylation of TBK1 (b) and the total levels of TBK1 (c); each dot represents one biological replicate. d, Summary of the results found in this study: toxic GOF mechanisms in C9orf72-ALS cause impairment of (endo)lysosomal homeostasis, at least in part mediated by alteration of TBK1 signalling, while C9orf72 LOF mainly influences endosome maturation