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Neuron-specific ablation of the Krabbe disease gene galactosylceramidase in mice results in neurodegeneration
Krabbe disease is caused by a deficiency of the lysosomal galactosylceramidase (GALC) enzyme, which results in the accumulation of galactosylceramide (GalCer) and psychosine. In Krabbe disease, the brunt of demyelination and neurodegeneration is believed to result from the dysfunction of myelinating...
Autores principales: | Kreher, Conlan, Favret, Jacob, Weinstock, Nadav I., Maulik, Malabika, Hong, Xinying, Gelb, Michael H., Wrabetz, Lawrence, Feltri, M. Laura, Shin, Daesung |
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Formato: | Online Artículo Texto |
Lenguaje: | English |
Publicado: |
Public Library of Science
2022
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Materias: | |
Acceso en línea: | https://www.ncbi.nlm.nih.gov/pmc/articles/PMC9255775/ https://www.ncbi.nlm.nih.gov/pubmed/35789331 http://dx.doi.org/10.1371/journal.pbio.3001661 |
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