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. Author manuscript; available in PMC: 2011 Sep 1.
Published in final edited form as: Nat Med. 2011 Feb 20;17(3):377–382. doi: 10.1038/nm.2313

Figure 1.

Figure 1

Mutant HTT triggers mitochondrial fragmentation, decrease in anterograde and retrograde transport, and neuronal cell death, which depends on polyQ length. (a) Fluorescence micrographs and ×6 zoom of boxed regions of neurons expressing HTT exon1-Q17, -Q46, or -Q97 and DsRed2-Mito. Scale bar, 50 µm. (b) Mitochondrial fragmentation of neurons expressing HTT exon1-Q17, -Q46, or -Q97 and DsRed2-Mito. (c) Cell death of neurons expressing HTT exon1-Q17, -Q46, or -Q97. (d) Fluorescence micrographs and ×3 zoom of boxed regions of MitoTracker Red stained human fibroblasts from an unaffected (left) or an adult onset HD individual (right). Scale bar, 25 µm. (e) Mitochondrial fragmentation in fibroblasts from an unaffected or HD individuals. (f) Kymographs of mitochondrial transport in neurons expressing HTT exon1-Q17, -Q46, or -Q97 and DsRed2-Mito. See also Movies S1, S2, S3. (g) Scatter plots of mitochondrial velocity in retrograde or anterograde direction as a function of distance traveled in 5 minutes in neurons (n = 10) expressing HTT exon1-Q17, -Q46, or -Q97 and DsRed2-Mito. (h) Anterograde and retrograde movement, motility, and mean velocity of mitochondria in the same neurons analyzed in (g). Data are mean ± s.e.m. of triplicate samples of representative experiments. Results are representative of three or more independent experiments. Statistics: one-way ANOVA test.