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Fig. 8 | Molecular Neurodegeneration

Fig. 8

From: Lentiviral vector-mediated overexpression of mutant ataxin-7 recapitulates SCA7 pathology and promotes accumulation of the FUS/TLS and MBNL1 RNA-binding proteins

Fig. 8

Immunoreactivity of TDP-43, FUS/TLS, MBNL1 and MBNL2 in the cerebellum of control and SCA7 patients. Representative immunohistochemically labelled cerebellar sections (counterstained with hematoxylin) from a SCA7 patient and a control with no neurological disease. a TDP-43 immunoreactivity was increased in the nucleus of PCs in the cerebellum of a SCA7 patient compared to control PCs where it remained diffuse with few TDP-43 dots. b Phosphorylated TDP-43 was strongly labeled in the nucleus of a PC of a SCA7 patient; only rare nuclear granules were observed in the nucleus of control PCs. c FUS/TLS shows increased immunoreactivity with FUS/TLS-positive small dots in the nucleus of a PC in a SCA7 patient compared to a control PC where FUS/TLS was almost not detectable in the nucleus. d Nuclear accumulation of the MBNL1 protein was higher in an atrophic PC in a SCA7 patient compared to PCs from a control. e MBNL2 diffuse immunoreactivity is increased in the nucleus and cytosol of a SCA7 PC, compared to control PCs where MBNL2 immunoreactivity is low in the cytoplasm and almost undetectable in the nucleus. Bars: 20 μm

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Molecular Neurodegeneration

ISSN: 1750-1326