Sensorimotor and Neurocognitive Dysfunctions Parallel Early Telencephalic Neuropathology in Fucosidosis Mice

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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Bibliographic Details
Main Authors: Stijn Stroobants, Heike Wolf, Zsuzsanna Callaerts-Vegh, Thomas Dierks, Torben Lübke, Rudi D’Hooge
Format: Article
Language:English
Published: Frontiers Media S.A. 2018-04-01
Series:Frontiers in Behavioral Neuroscience
Subjects:
lysosomal storage disorder
fucosidosis
mouse model
neuropathology
behavior
motor function
Online Access:http://journal.frontiersin.org/article/10.3389/fnbeh.2018.00069/full

Internet

http://journal.frontiersin.org/article/10.3389/fnbeh.2018.00069/full

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