Rescue of motoneuron degeneration by small molecules in ATXN3-CAG89 transgenics. Rescue ofmotoneurondegeneration by small molecules in ATXN3-CAG89 transgenics.

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Rescue of motoneuron degeneration by small molecules in ATXN3-CAG89 transgenics. Rescue ofmotoneurondegeneration by small molecules in ATXN3-CAG89 transgenics. Shown are representative photos of living, adult unc-47p::mCherry; ATXN3-CAG89 transgenics at day 5 of adulthood with or without compounds. Images are black and white, photo-reversed to aid visualization of neurons. (A) Image of degenerating GABAergic motoneurons from an entire ATXN3-CAG89 transgenic. The panel on the right is a magnification of the area indicated by the rectangle. Arrows indicate gaps or breaks along neuronal processes. Rescue of neurodegeneration was observed in ATXN3-CAG89 worms in the presence of (B) 60 μM MB, (C) 50 µM Sal or (D) 50 µM Gua. (E) Quantification of neurodegeneration in transgenic ATXN3-CAG89 worms at day 5 of adulthood. Significant rescue of the neurodegeneration morphology was observed in ATXN3-CAG89 transgenics when treated with 60 μM MB (*P<0.05), 50 µM Sal (***P<0.001) or 50 µM Gua (****P<0.0001) (by Student's unpaired t-test, n=100 for each condition). These experiments were repeated three times. Gua, guanabenz; MB, Methylene Blue; Sal, salubrinal. Yasmin Fardghassemi et al. Dis. Model. Mech. 2017;10:1465-1480 © 2017. Published by The Company of Biologists Ltd