Amyloidosis, Synucleinopathy, and Prion Encephalopathy in a Neuropathic Lysosomal Storage Disease: The CNS-Biomarker Potential of Peripheral Blood
Figure 3
Gene dysregulation involved in synucleinopathy in the brain and blood in MPS IIIB mice and their response to rAAV9-hNAGLU gene delivery.
a & b: Total RNA from the brain (cortex), peripheral blood (PB) and control somatic tissues of 6 mo old mice were assayed for Snca (a) and Park2 (b) by qRT-PCR (n = 9–12/group). Data: relative expression vs. wt. Brain tissue sections (4 µm) from 6 mo-old mice (n = 7) were assayed by IHC for Snca (c) and Park2 (d), positive cells/signals were stained brown (d). Snca staining intensity was quantitated using ImageJ (c). +/+: wt mice; −/−: non-treated MPS IIIB mice; AAV9: rAAV9-treated MPS IIIB mice; CA3: hippocampus CA3 region; DG: dentate gyrus; Py: pyramidal cell layer of hippocampus; red arrows: Park2-positive neuronal cell bodies; black arrows: Park2-positive neuronal processes. *: P<0.05 vs. wt; #: P<0.05 vs. AAV9-treated; +: P>0.05 vs. wt; $: P>0.05 vs.AAV9-treated. Scale bar: 50 µm.