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Fig. 3. Pharmacological inhibition of HDAC6 rescues abnormalities in the mitochondrial network in ALS patient-derived fibroblasts carrying the RAPGEF2-E1357K variant. (A) Confocal images of Mito-RFP-expressing control and patient (E1357K) fibroblasts treated with DMSO or 1 µM tubastatin A (Tub A). Cells were labeled with anti-acetylated α-tubulin (Ac-tub) and DAPI. Bottom panels show higher magnification views of the regions marked by boxes. Scale bar, 5 µm. (B and C) Quantification of mitochondria length and cell size (n=7 for DMSO-treated control fibroblasts; n=6 for DMSO-treated patient fibroblasts (E1357K); n=11 for Tub A-treated control fibroblasts; n=11 for Tub A-treated patient fibroblasts (E1357K). Data are presented as mean±SEM. Comparisons are made with DMSO-treated control fibroblasts (*p<0.001).
Exp Neurobiol 2018;27:550~563
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