Fig. 4
From: Mutant UBQLN2P497H in motor neurons leads to ALS-like phenotypes and defective autophagy in rats
Denervation atrophy of skeletal muscles in ChATtTA/UBQLN2P497H rats. a-d H&E staining shows groups of atrophic muscle fibers (a, arrows) and ectopic nuclei (c, arrows) in the gastrocnemius muscles of ChATtTA/UBQLN2P497H (P497H) but not in ChATtTA single transgenic (ChATtTA) rats at 12 months old. e-h nonspecific esterase staining shows the grouped muscle atrophy (e, g, arrows) in the gastrocnemius muscles of P497H rats, and which are more severe atrophy in 12-month old rats than 6 months, but not in ChATtTA rats. The red arrows point to the neuromuscular junctions (NMJ). i-l The projection of confocal images reveals the innervation of NMJs in gastrocnemius muscles. The arrows (j-l: P497H) show that the motor end-plates are poorly innervated in P497H rats compared to ChATtTA rats (I), and the neurofilaments progressively form inclusions accompanied with the denervation of NMJ in P497H rats from 3 months to 12 months old. Scale bars: 100 μm (a-b, e-h), 30 μm (c, d), 20 μm (i-l)