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 age. At pr...

Disgrifiad llawn

Wedi'i Gadw mewn:
Manylion Llyfryddiaeth
Cyhoeddwyd yn:Int J Mol Sci
Prif Awduron: Belleri, Mirella, Coltrini, Daniela, Righi, Marco, Ravelli, Cosetta, Taranto, Sara, Chiodelli, Paola, Mitola, Stefania, Presta, Marco, Giacomini, Arianna
Fformat: Artigo
Iaith:Inglês
Cyhoeddwyd: MDPI 2019
Pynciau:
Article
Mynediad Ar-lein:https://ncbi.nlm.nih.gov/pmc/articles/PMC6982065/
https://ncbi.nlm.nih.gov/pubmed/31905906
https://ncbi.nlm.nih.govhttp://dx.doi.org/10.3390/ijms21010251
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