Figure 11.

Comparison of twi-5J and twitcher clinical symptoms and disease progression. (A) Clinical symptoms of Krabbe disease mouse models. (B) Schematic comparing characteristics of disease progression between twi-5J and twitcher in the CNS and PNS. In the forebrain, axonal pathology is observed in twi-5J but not in twitcher. In contrast, significant axonal pathology is not detected in the spinal cord of twi-5J. The twi-5J sciatic nerve exhibits early perturbation in myelination, normalization of g-ratios and MBP expression by P15, and severe dysmyelination by P25. Inflammatory cytokine progression in the spinal cord and sciatic nerve has not been examined in twitcher (56,70). A comparison of psychosine accumulation is presented in Figure 10.