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A
Expression of ATXN3tr‐Q78 in Fuz
−/− HEK293 cells showed reduced cell death. The parental HEK293 cells were used as control. Knockout of Fuz did not alter relative cell death in the untransfected or ATXN3tr‐Q27 cells, indicating that Fuz knockout did not induce cell death. Error bars represent s.e.m., n = 3. Statistical analysis was performed using one‐way ANOVA followed by post hoc Tukey's test. **P < 0.01.
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B
The JNK phosphorylation and caspase‐3 cleavage induced by ATXN3tr‐Q78 were suppressed in Fuz
−/− HEK293 cells. The parental HEK293 cells were used as control. n = 3.
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C
Knockdown of Fuz expression suppressed JNK phosphorylation and caspase‐3 cleavage in ATXN3tr‐Q78‐expressing rat primary cortical neurons. n = 3.
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D
Knockdown of Fuz expression suppressed the activation of JNK and caspase‐3 in SCA3 patient fibroblasts. n = 3.
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E, F
(E) Knockdown of fy expression rescued neurodegenerative eye phenotype in SCA3 transgenic flies. The integrity of rhabdomeres in ATXN3fl‐Q27 flies was not affected upon fy knockdown. The flies were of genotypes w; gmr::Gal4 UAS::ATXN3fl‐Q27/+; +/+, w; gmr::Gal4 UAS::ATXN3fl‐Q27/UAS::fy‐dsRNA
KK100638
; +/+, w; gmr::Gal4/+; UAS::ATXN3fl‐Q84/+ and w; gmr::Gal4/UAS::fy‐dsRNA
KK100638
; UAS::ATXN3fl‐Q84/+. Scale bars: 5 μm. (F) is the quantification of (E). Error bars represent s.e.m., n = 3. For every control or experimental group, a total of 200 ommatidia collected from 20 fly eyes were examined in each replicate. Statistical analysis was performed using one‐way ANOVA followed by post hoc Tukey's test. *P < 0.05, ***P < 0.001.
Data information: Beta‐tubulin was used as loading control.
represents the number of biological replicates. Only representative images and blots are shown.
