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Atg4b-Dependent Autophagic Flux Alleviates Huntington’s Disease Progression

Figure 4

CStS recapitulate MSNs degeneration observed in (CAG)150-het mouse model.

A) Single confocal planes for the immunohistochemistry of CStS derived from (CAG)150-het mice show progressive mHtt accumulation in the striatum at DIV14 and DIV21. B) Higher magnification of MSNs in (CAG)150-het slices show selective mHtt accumulation at DIV21. Note the extranuclear mHtt accumulation and few small nuclear inclusions (arrows). C) Time course quantification of mHtt intensity per area shows progressive accumulation in (CAG)150-het slices. D) Western blot showing mHtt is selective to (CAG)150-het slices. Biochemical detection of soluble mHtt in (CAG)150-het slices; each lane represents an aliquot of 10 µg from total lysates of distinct slices. N = 10 images from 5 independent slices; median values ± SEM; ***p<0.001 Bars: (A) 30 µm, (B) 10 µm.

Figure 4

doi: https://doi.org/10.1371/journal.pone.0068357.g004