Fig. 6: Impaired myelin integrity in Cln3∆ex7/8/∆ex7/8 mice.
From: Loss of CLN3 in microglia leads to impaired lipid metabolism and myelin turnover
Representative brain sections from 20-month-old wild-type (a, b) and Cln3∆ex7/8/∆ex7/8 (c, d) mice immunostained for myelin using anti-MBP (red) antibody. Hoechst nuclear counterstaining is shown in blue. Scale bar = 500 μm. The bar graph represents the average of mean MBP intensities (e). N = 9 brain sections (3 mice per genotype, 3 brain sections per mouse, matched to represent comparable brain regions for control and Cln3∆ex7/8/∆ex7/8). Data are represented as mean ± standard error of the mean (SEM); ****P ≤ 0.0001, Student’s t-test. f Representative electron micrographs of transverse sections through the optic nerves from 18-months-old wild-type and Cln3-deficient mice. Scale bar= 2 µm. g Representative examples of distinct structural aberrations in myelin sheaths of Cln3-deficient mice. Myelin infoldings or outfoldings (arrowheads), redundant myelin (arrows), myelin fragments (hashtags), and myelin vacuoles (circles) are frequent. Occasionally, microglial cells (MG, green pseudocolor) containing lysosomal storage material (asterisks) can be detected. h Quantification of fibers with cumulative myelin aberrations (top) and amyelinated or thinly myelinated fibers (bottom) in the optic nerves from 18-months-old wild-type and Cln3-deficient mice (n = 5 mice per group, two-sided unpaired t test. top: t = 10.35, d.f. = 8, bottom: t = 10.35, d.f. = 8). Data are presented as the mean ± s.d.
