Figure 5. Spongiform degeneration of brain and DRG is rescued by the Fig4-I41T transgene.

Top: Sagittal brain section, cortex, and DRG from Fig4 null mice demonstrating enlarged ventricle and spongiform degeneration (scale bar = 200 µm). Middle panels: Partial rescue of Fig4 null mice carrying Tg705 (2× expression) and Tg721 (5× expression). Bottom panel: Sagittal sections of brain from wildtype mice (left) and transgenic mice in the final stage of disease progression. Both transgenes provide protection from neurodegeneration, with greater protection in the higher expressing line.