Dysregulated autophagy as a new aspect of the molecular pathogenesis of Krabbe disease
Krabbe disease (KD) is a childhood leukodystrophy with no cure currently available.KD is due to a deficiency of a lysosomal enzyme called galactosyl-ceramidase (GALC) and is characterized by the accumulation in the nervous system of the sphingolipid psychosine (PSY), whose cytotoxic molecular mechanism is not fully known yet.Here, we study the expr