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 2

Generation of CMT2F-specific in vitro model by differentiation of patient-derived iPSCs into MNs. (a) Schematic presentation of MN differentiation. (b) Differentiated MNs showed typical cellular morphology (original magnification, 50x). Scale bars: 200 μm. (c) S135F-MNs and P182L-MNs expressed MN-specific transcription factors such as HB9 and ISL1/2; neuronal cytoskeletal markers such as TuJ1, SMI32, and MAP2; ChAT; and the synaptic vesicular marker synapsin (original magnification, 400x and 600x). Scale bars: 100 μm. (d) Differentiation efficiency of S135F-MNs and P182L-MNs was comparable to control MNs in terms of neuronal marker expression such as SMI32/DAPI and MAP2/DAPI (WA09-MNs; , hFSiPS1-MNs; , S135F-MNs; , and P182L-MNs; ). (e) Axonal length of S135F-MNs was comparable to that of control MNs. Axonal length was measured by culturing fully differentiated MNs in microchannel plates for an additional 2 weeks (WA09-MNs: and S135F-MNs: ).

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