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. 2017 Oct 11;8:861. doi: 10.1038/s41467-017-00911-y

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© The Author(s) 2017

Open Access This article is licensed under a Creative Commons Attribution 4.0 International License, which permits use, sharing, adaptation, distribution and reproduction in any medium or format, as long as you give appropriate credit to the original author(s) and the source, provide a link to the Creative Commons license, and indicate if changes were made. The images or other third party material in this article are included in the article’s Creative Commons license, unless indicated otherwise in a credit line to the material. If material is not included in the article’s Creative Commons license and your intended use is not permitted by statutory regulation or exceeds the permitted use, you will need to obtain permission directly from the copyright holder. To view a copy of this license, visit http://creativecommons.org/licenses/by/4.0/.

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Fig. 5 — Restoration of axonal transport and of ER-mitochondrial overlay by HDAC6 inhibition. a Immunostaining for ER (using mouse PDI antibody) and mitochondria (using rabbit TOM-20 antibody) of motor neurons derived from iPSC from patients carrying the P525L mutation and healthy controls before and after an overnight treatment with ACY-738 (1 μM). The separate views show co-localized pixels in the cell body and neurites. Scale bar = 5 µm. b Quantification of the proportion of ER-merged regions and mitochondrial-merged regions relative to the total mitochondrial staining. Data were analyzed by determining the Pearson’s correlation coefficient using ImageJ (n = 20, mean ± SEM, one-way ANOVA with post-hoc Tukey’s test, *, ***P values of 0.05 and 0.001). c Phosphatidylcholine (PC) level in culture media from motor neuron. Media were taken for ELISA after 2 days on the culture at the fourth week after the start of differentiation. A decrease was observed in patient-derived motor neurons. Representative experiment is shown (mean of technical duplicates ± SD). d Quantification of stationary mitochondria, moving mitochondria, and ratio between moving to total mitochondria normalized to a neurite length of 100 µm in motor neurons derived from patient and healthy control iPSC lines at the fourth week after plating with or without an overnight treatment with ACY-738 (n = 18, n = 16, n = 15, n = 18, n = 15, n = 13, n = 17, n = 13 for Ctr1 + DMSO, Ctr1 + ACY-738, Ctr2 + DMSO, Ctr2 + ACY-738, R521H + DMSO, R521H + ACY-738, P525L + DMSO, P525L + ACY-738, respectively, mean ± SEM, one-way ANOVA with post-hoc Tukey’s test; *, **P values of 0.05 and 0.01, respectively). e Quantification of stationary ER vesicles, moving ER vesicles, and ratio between moving to total vesicles normalized to a neurite length of 100 µm in motor neurons derived from patient and healthy control iPSC lines at the fourth week after plating with or without an overnight treatment with ACY-738 (n = 10, n = 19, n = 11, n = 18, n = 12, n = 12, n = 11, n = 16 for Ctr1 + DMSO, Ctr1 + ACY-738, Ctr2 + DMSO, Ctr2 + ACY-738, R521H + DMSO, R521H + ACY-738, P525L + DMSO, P525L + ACY-738, respectively, mean ± SEM, one-way ANOVA with post-hoc Tukey’s test; *, **, ***P values of 0.05, 0.01, and 0.001, respectively)