Early axonal loss accompanied by impaired endocytosis, abnormal axonal transport, and decreased microtubule stability occur in the model of Krabbe’s disease

In Krabbe’s Disease (KD), a leukodystrophy caused by β-galactosylceramidase deficiency, demyelination and a myelin-independent axonopathy contribute to the severe neuropathology. Beyond axonopathy, we show that in Twitcher mice, a model of KD, a decreased number of axons both in the PNS and CNS, and...

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Bibliografski detalji
Izdano u:Neurobiol Dis
Glavni autori: Teixeira, Carla Andreia, Miranda, Catarina Oliveira, Sousa, Vera Filipe, Santos, Telma Emanuela, Malheiro, Ana Rita, Solomon, Melani, Maegawa, Gustavo H., Brites, Pedro, Sousa, Mónica Mendes
Format: Artigo
Jezik:Inglês
Izdano: 2014
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Article
Online pristup:https://ncbi.nlm.nih.gov/pmc/articles/PMC4307018/
https://ncbi.nlm.nih.gov/pubmed/24607884
https://ncbi.nlm.nih.govhttp://dx.doi.org/10.1016/j.nbd.2014.02.012
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