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...

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Detalles Bibliográficos
Autores principales: Belleri, Mirella, Coltrini, Daniela, Righi, Marco, Ravelli, Cosetta, Taranto, Sara, Chiodelli, Paola, Mitola, Stefania, Presta, Marco, Giacomini, Arianna
Formato: Online Artículo Texto
Lenguaje:English
Publicado: MDPI 2019
Materias:
Article
Acceso en línea:https://www.ncbi.nlm.nih.gov/pmc/articles/PMC6982065/
https://www.ncbi.nlm.nih.gov/pubmed/31905906
http://dx.doi.org/10.3390/ijms21010251

Internet

https://www.ncbi.nlm.nih.gov/pmc/articles/PMC6982065/
https://www.ncbi.nlm.nih.gov/pubmed/31905906
http://dx.doi.org/10.3390/ijms21010251

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