The Smn-Independent Beneficial Effects of Trichostatin A on an Intermediate Mouse Model of Spinal Muscular Atrophy
Figure 3
TSA administration decreases neuromuscular junction loss in Smn2B/- mice.
(A) Confocal micrographs show neuromuscular junctions (NMJs) labelled with antibodies against neurofilament (NF, red), synaptic vesicle protein 2 (SV2, red) and alpha-bungarotoxin (BTX, green). Note that whilst there is widespread NMJ pathology in vehicle treated mice, as evidence by full/partial denervation (white arrowhead/arrow respectively), NF accumulation (yellow arrow) and small, immature post-synaptic endplates, NMJs appear much more healthy in TSA treated mice, as evidenced by a decrease in denervation and NF accumulation, and an increase in post-synaptic size and maturity (scale bar = 50 µm). (B, C) Bar charts show an increase in the percentage of fully occupied endplates in both the transversus abdominis (TVA; B) and rectus abdominis (RA; C) muscles following TSA treatment. * P<0.05 by Mann Whitney test.