Dysregulated autophagy as a new aspect of the molecular pathogenesis of Krabbe disease

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 mecha...

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Bibliographic Details
Main Authors: Ambra Del Grosso, Lucia Angella, Ilaria Tonazzini, Aldo Moscardini, Nadia Giordano, Matteo Caleo, Silvia Rocchiccioli, Marco Cecchini
Format: Article
Language:English
Published: Elsevier 2019-09-01
Series:Neurobiology of Disease
Subjects:
Globoid cell leukodystrophy
GLD
Krabbe disease
KD
Autophagy
Psychosine
Online Access:http://www.sciencedirect.com/science/article/pii/S0969996119301238

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http://www.sciencedirect.com/science/article/pii/S0969996119301238

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