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

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 contributes 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 in...

Full description

Bibliographic Details
Main Authors: Carla Andreia Teixeira, Catarina Oliveira Miranda, Vera Filipe Sousa, Telma Emanuela Santos, Ana Rita Malheiro, Melani Solomon, Gustavo H. Maegawa, Pedro Brites, Mónica Mendes Sousa
Format: Article
Language:English
Published: Elsevier 2014-06-01
Series:Neurobiology of Disease
Subjects:
Krabbe's disease
Twitcher
Leukodystrophy
Myelin
Axonal transport
Endocytosis
Online Access:http://www.sciencedirect.com/science/article/pii/S0969996114000540

Internet

http://www.sciencedirect.com/science/article/pii/S0969996114000540

Similar Items