Research Article

Accumulation of Misfolded SOD1 in Dorsal Root Ganglion Degenerating Proprioceptive Sensory Neurons of Transgenic Mice with Amyotrophic Lateral Sclerosis

Figure 6

(a) Semithin plastic section of a DRG from end-stage (P120) mice showing a degenerating, microvacuolated, large neuronal soma, which is surrounded by apparently normal medium-sized neuronal cells. ((b) and (c)) The cells indicated in the semithin sections were examined by electron microscopy which revealed a well-preserved organelle structure in the medium-sized neuronal cell (b) and extensive microvacuolization in the degenerating large soma (c). (d) A large myelinated axonal profile adjacent to the DRG sensory neurons shows an accumulation of highly vacuolated mitochondria with a comparable morphology to that of degenerating mitochondria typically described in motor axons of mice (as shown in (e), taken from a sample of facial nucleus). Scale bars: 20 μm in (a), 0.5 μm in (b) and (c), and 1.5 μm in (d) and (e).
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