Atg4b-Dependent Autophagic Flux Alleviates Huntington’s Disease Progression
Figure 1
MSNs degeneration in R6/2 mice.
A) Immunohistochemistry against DAPI (blue) and mHtt (green) of R6/2 brain sections shows progressive mHtt accumulation in striatum. B) Quantification of mHtt staining from A) showing mHtt aggregation in terms of density (left) and size (right). R6/2 mice develop progressive mHtt aggregation. C) Progressive striatal neurodegeneration in R6/2 mice. Brain sections from adult R6/2 mice and WT controls were stained for a striatal marker DARPP-32 (red), neurofilament (NeuF, green) and NeuN (green) at 10 weeks of age. By this time the R6/2 mice have developed mHtt accumulation and they have decreased DARPP-32 staining as well as NeuF, whereas the neuronal marker NeuN is unchanged. D) Left: the ratio of striatal NeuN and DAPI positive nuclei is unchanged between WT and R6/2 mice at 10 weeks of age, in line with the limited cell death characteristic of the R6/2 mouse model. Right: quantification of C) shows a highly significant decrease in both NeuF and DARPP-32 intensity. Three mice per condition; N = 5 images/each; median values ± SEM; Students t test:*p<0.05, **p<0.01 Bars and ***p<0.001: (A) 200 µm, (C) 50 µm.
