Supplementary Figure 3: Depletion of lysosomal enzymes in tissues from CLN8-deficient mice.
From: CLN8 is an endoplasmic reticulum cargo receptor that regulates lysosome biogenesis
(a), Immunohistochemistry of lysosomal proteins in liver and brain from WT and CLN8-deficient mice. Liver and brains were collected from 2-month-old Cln8mnd and WT mice. CTSD and TPP1 protein levels are strongly reduced in CLN8-deficient mice, compared to WT, in all tested conditions. LAMP1 immunostaining is unchanged. Scale bar, 100 μm. (b), Confocal microscopy of lysosomal enzymes (red) and LAMP1 (green) on brain sections of WT and CLN8-deficient mice. Pearson correlation showed a significant reduction of the enzyme/LAMP1 signal overlap in CLN8-deficient mice compared to WT mice. Insets show four-fold magnification. Scale bar: 20 μm. Images in (a) are representative of n = 3 independent experiments. In (b), data are means ± SEM (n = 3 independent experiments, n = 10 independent images quantified, **P < 0.01, ****P < 0.0001, two-tailed Student’s t-test)
