Figure 7.

AAV9/JeT-GAN Gene Therapy Improves IF Cytoskeleton in GAN Peripheral Nerves

EM examination of sciatic nerves from 24-month-old control GAN/Y KO and AAV9/JeT-FLAG-GAN-injected GAN/Y KO mice at 6 months post-IT-injection (cohort 4). (A–C) Control GAN KO mouse and (D–F) GAN-treated GAN KO mouse. Stars indicate intact unmyelinated fibers and associated Schwann cells, arrows indicate normal Schwann cell cytoplasm associated with myelinated fibers, asterisks indicate dense, disorganized accumulations of NFs in fibers, arrowhead indicates increased density of NFs in myelinated fiber, and crosses indicate accumulation of IFs in Schwann cell cytoplasm associated with myelinated fibers. Representative images from n = 6 per group. Scale bar: 5 μm (A and D), 1 μm (B and E), and 0.5 μm (C and F).