Figure 1

Lysosomal dysfunction and increased α-synuclein concentrations in CLN6 sheep. (A) Cathepsin D activity was measured in homogenates (1 μg) isolated from the occipital lobe of 3, 7 and 14 month old control or CLN6 affected Merino sheep (N = 3 per group) using a fluorometric Cathepsin D activity assay. (B-D) Densitometry and representative immunoblots of homogenates (5–40 μg) isolated from the occipital lobe of 3, 7 and 14 month old control or CLN6 affected sheep probed with antibodies directed against V-ATPase (B), α-synuclein (C) or ATP13a2 (D). GAPDH was used as a loading control. Quantification was performed in ImageJ and concentrations are expressed relative to those in control sheep at each age. Data are mean + SEM. **p < 0.01, ***p < 0.001 by Student’s t test. C, control.