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Fig. 7 | Acta Neuropathologica Communications

Fig. 7

From: Cryptic amyloidogenic elements in mutant NEFH causing Charcot-Marie-Tooth 2 trigger aggresome formation and neuronal death

Fig. 7

mutant NEFH aggregate in ovo and trigger apoptosis. a confocal images of chick embryo spinal cord cryosection after in ovo electroporation. Sections of 20 μm were counterstained for beta3-tubulin (TUJ1 clone), a neuronal specific isoform. Scale bar represent 100 μm. b Zoom of the anterior horn of the spinal cord to visualize mutant NEFH aggregates in the soma of electroporated neurons, which co localize with p62/SQSTM1 and LC3b. Scale bar: 10 μm. c Caspase 3 activated staining of the spinal cords appears in some neurons harboring mutant NEFH protein aggregates. Scale bar represent 20 μm. Arrow show apoptotic cells. d Quantification of caspase 3 activation in electroporated neurons. Values represent means of percentage +/− sem from at least 20 electroporated 20 μm thick cryosections of four different embryos per condition and analyzed by one way ANOVA followed by multiple comparisons versus control group (Dunnett’s Method) * p < 0.001

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