Proteostasis network alteration in lysosomal storage disorders: insights from the mouse model of Krabbe diseaseProteostasis network alteration in lysosomal storage disorders: insights from the mouse model of Krabbe diseaseProteostasis network alteration in lysosomal storage disorders: insights from the mouse model of Krabbe disease

Krabbe disease is characterized by widespread demyelination due to cell death by apoptosis, mainly in oligodendrocytes and Schwann cells. In this study, we highlight that in the brain of 6 days old twitcher mice (model of Krabbe disease) there is evidence of impairment in protein synthesis and degradation, as well as in cytoskeleton remodeling, with a lamin meshwork disaggregation and clear evidence of apoptosis, also demonstrated in oligodendrocytes. Even if further studies are needed to address the mechanisms behind this pathology, this study provide interesting insights on the early onset of Krabbe disease that may be useful for developing of innovative pharmacological treatment.