Extended Data Fig. 3: Preservation of neuromuscular junctions (NMJs) in gastrocnemius muscle in SOD1G37R mice treated before or after disease onset with AAV9-shRNA-SOD1.

a-c, Several normally-appearing NMJs triple-stained with BTX/SYN/NF-H and which are similar to (a) wilde-type nontransgenic animals can be seen in (c) SOD1G37R animals treated with AAV9-shRNA-SOD1 at age of 120 days and analyzed at age ~470 days. b, Denervated (BTX + /SYN + , but NF-H negative) NMJs are found in sham-operated SOD1G37R animals. d,e, Compared to wild-type nontransgenic animal (d) a partial denervation (loss of BTX + /SYN + /NF-H + ) is seen in SOD1G37R animals at age of ~348 days (e). f,g, Compared to end stage sham-operated animals which show extensive loss of NMJs (f) the innervation is maintained in SOD1G37R animals treated after disease onset (at age ~348 days) with AAV9-shRNA-SOD1 and analyzed at age between 398-465 days (g). A representative images from at least 3 individual animals per experimental group are shown.