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. 2019 Dec 10;11:1759091419886212. doi: 10.1177/1759091419886212

Figure 4.

Figure 4.

Muscle atrophy, muscle fiber switching and ultrastructural abnormalities in BACHD. (a and b) Representative images of TA skeletal muscle fibers from 12-month-old WT and BACHD mice. Scale bar: 50 μm. (c to c’’’) and (d to d’’’) Representative images of TA fiber typing from 12-month-old WT and BACHD mice. Scale bar: 50 μm. (e to j) Representative electron micrographs of TA fibers from WT and BACHD animals. Observe a normal triad in WT (e, yellow box). (g) High-magnification view of the area in (f) showing marked glycogen accumulation in the intermyofibrillar spaces (red arrows), sarcoplasmic reticulum enlargement (yellow asterisk) and Z-line discontinuity (blue arrows) in BACHD animals. (h to j) Observe profound mitochondrial changes (green arrows). Scale bar: 500 nm. We analyzed 90 images per genotype from six individual animals (three per genotype). (k) Quantitative analysis shows the CSA mean values for WT and BACHD TA muscle fibers. These results represent the mean ± SD of more than 4.000 muscle fibers per genotype (*p < .04; unpaired Student’s t test; n = 3 animals per genotype). (l) Quantitative analysis of the fiber typing showing decreased number of IIB isoform and increase of IIX in BACHD TA muscle fibers compared with WT (*p = .01 and *p = .02; unpaired Student’s t test; n = 3 animals per genotype). (m) Quantitative analysis of the CSA from fiber typing (*p = .03; unpaired Student’s t test; n = 3 animals per genotype). The results represent the mean ± SD (unpaired Student’s t test, *p < .05; n = 3 animals per genotype). CSA = cross-sectional area; WT = wild type.