Figure 2

CTB tracing and Golgi fragmentation in motor neurons with ubiquitinated SOD1-inclusions. A -C) Double-labeling confocal image of CTB and ubiquitinated epitopes showing a CTB-positive motor neuron with a large inclusion in the proximal dendrite (A' and A''), a CTB-positive motor neuron with intense ubiquitin labeling throughout most of the somato-dendritic compartment (B' and B''''), and ubiquitin-labeling in distal CTB-positive dendrites (C-C'''). D) Double-labeling confocal image of GM130 and misfolded SOD1 (SEDI epitope) showing a motor neuron with fragmented Golgi and a large SEDI-immunoreactive inclusion. E-F) Confocal image showing motor neurons labeled for ChAT with fragmented Golgi apparatus and poly-ubiquitinated epitope (arrow) in L4 lumbar spinal cord section of 20 (E) and 35 (F) weeks old T3hSOD1 mouse. G) Bar graph showing the number of Golgi fragmented motor neurons in L4 spinal cord sections of G1del versus T3hSOD1 mice. Values represent Means ± SE from 3 mice per group. Note that no motor neurons with fragmented Golgi occur in non-transgenic, T3 and hSOD1 mice at 35 weeks of age. Bars: 50 μm (A-C), 10 μm (D-F).