β-Galactosylceramidase Deficiency Causes Bone Marrow Vascular Defects in an Animal Model of Krabbe Disease

β-Galactosylceramidase Deficiency Causes Bone Marrow Vascular Defects in an Animal Model of Krabbe Disease

Krabbe disease (KD) is an autosomal recessive sphingolipidosis caused by the deficiency of the lysosomal hydrolase β-galactosylceramidase (GALC). Oligodendroglia degeneration and demyelination of the nervous system lead to neurological dysfunctions which are usually lethal by two years of a...

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Bibliographic Details
Main Authors: Mirella Belleri, Daniela Coltrini, Marco Righi, Cosetta Ravelli, Sara Taranto, Paola Chiodelli, Stefania Mitola, Marco Presta, Arianna Giacomini
Format: Article
Language:English
Published: MDPI AG 2019-12-01
Series:International Journal of Molecular Sciences
Subjects:
bone marrow
endothelium
beta-galactosylceramidase
krabbe disease
<i>twitcher</i> mice
Online Access:https://www.mdpi.com/1422-0067/21/1/251

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https://www.mdpi.com/1422-0067/21/1/251

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