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. Author manuscript; available in PMC: 2017 Aug 18.
Published in final edited form as: Mol Cell. 2016 Aug 4;63(4):608–620. doi: 10.1016/j.molcel.2016.06.030

Figure 1. Coq8a−/− Mice Develop Cerebellar Ataxia and Mild Exercise Intolerance.

Figure 1

(A) Immunoblot analysis of COQ8A abundance in 30-week-old mice.

(B) Accelerating rotarod retention times of 10-week-old mice (mean ± SEM, n = 8–12). ANOVA test P<0.05.

(C) Left, representative footprints of 10-week-old mice. Right, “linearity coefficient”, which is proportional to non-linear movement (mean ± SEM, n = 8–12), *P<0.05, **P<0.01.

(D) Number of hind limb coordination errors (mistakes) by mice on beam test (mean ± SEM, n = 8–12), *P<0.05, **P<0.01, ***P<0.01.

(E) Hematoxylin and eosin (H&E) (upper) and Calbindin (lower) staining of cerebellar sections of 15- and 40-week-old mice, respectively. *Shrunken Purkinje neurons; scale bar, 20 μm.

(F) Electron microscopy of 30-week-old cerebella. G, Golgi apparatus; *dilated Golgi; scale bars, 5 μm upper, 1 μm lower.

(G) Electrophysiological recordings of mouse Purkinje cells. ISI and CV2 (mean ± SEM) (n = 3, > 40 cells/mouse); ** Mann-Whitney U test P<0.01.

(H) Maximum speed (upper) and duration (lower) on treadmill test by 10-month-old mice (mean ± SEM, n = 8), *P<0.05.

(I) H&E stained skeletal muscle sections of 7-month-old mice. Scale bar, 50 μm.

(J) Electron microscopy of quadriceps showing broken mitochondria with collapsing cristae (arrows). Upper: 7-month-old mice, scale bar, 2 μm. Lower: 15-month-old mice, scale bar, 1 μm.

See also Figure S1.