CNS Penetration of Intrathecal-Lumbar Idursulfase in the Monkey, Dog and Mouse: Implications for Neurological Outcomes of Lysosomal Storage Disorder
Figure 7
Electron micrographs of brain cells in uninjected and IT-injected (3 doses) I2S knockout mice.
Pathological improvements occurred at the ultrastructural level. (A) Neurons of uninjected mice had lamellated inclusions, zebra body-like structures and vacuoles containing granular storage material (insert), which was reduced in I2S injected mice (B). Oligodendrocytes of uninjected mice showed large electron-lucent storage vacuoles (arrow; C) while oligodendrocytes of I2S-injected mice had minimal vacuolation (D).