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...

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Detalles Bibliográficos
Autores principales: Kreher, Conlan, Favret, Jacob, Weinstock, Nadav I., Maulik, Malabika, Hong, Xinying, Gelb, Michael H., Wrabetz, Lawrence, Feltri, M. Laura, Shin, Daesung
Formato: Online Artículo Texto
Lenguaje:English
Publicado: Public Library of Science 2022
Materias:
Research Article
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

Internet

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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