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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...
Autores principales: | Belleri, Mirella, Coltrini, Daniela, Righi, Marco, Ravelli, Cosetta, Taranto, Sara, Chiodelli, Paola, Mitola, Stefania, Presta, Marco, Giacomini, Arianna |
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Formato: | Online Artículo Texto |
Lenguaje: | English |
Publicado: |
MDPI
2019
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Materias: | |
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 |
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