HDAC6 Inhibitors Rescued the Defective Axonal Mitochondrial Movement in Motor Neurons Derived from the Induced Pluripotent Stem Cells of Peripheral Neuropathy Patients with HSPB1 Mutation

Figure 5

HDAC6 inhibitors reversed axonal defect of mitochondrial transport by increasing acetylation of -tubulin. (a and b) Western blot assay showed that treatment of HDAC6 inhibitors increased acetylated -tubulin levels in S135F-MNs and P182L-MNs (paired -tests, and ). (c) HDAC6 inhibitors increased the absolute velocity of mitochondrial movements in S135F-MNs and P182L-MNs (WA09-MNs; , S135F-MNs; , and P182L-MNs; ). (d) HDAC6 inhibitors increased the percentage of moving mitochondria in S135F-MNs and P182L-MNs (WA09-MNs; , S135F-MNs; , and P182L-MNs; ). Unpaired -tests, , , and .

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