Sensorimotor and Neurocognitive Dysfunctions Parallel Early Telencephalic Neuropathology in Fucosidosis Mice

Fucosidosis is a lysosomal storage disorder (LSD) caused by lysosomal α-L-fucosidase deficiency. Insufficient α-L-fucosidase activity triggers accumulation of undegraded, fucosylated glycoproteins and glycolipids in various tissues. The human phenotype is heterogeneous, but progressive motor and cog...

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Detalhes bibliográficos
Publicado no:Front Behav Neurosci
Main Authors: Stroobants, Stijn, Wolf, Heike, Callaerts-Vegh, Zsuzsanna, Dierks, Thomas, Lübke, Torben, D’Hooge, Rudi
Formato: Artigo
Idioma:Inglês
Publicado em: Frontiers Media S.A. 2018
Assuntos:
Neuroscience
Acesso em linha:https://ncbi.nlm.nih.gov/pmc/articles/PMC5906539/
https://ncbi.nlm.nih.gov/pubmed/29706874
https://ncbi.nlm.nih.govhttp://dx.doi.org/10.3389/fnbeh.2018.00069
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