A mouse model for fucosidosis recapitulates storage pathology and neurological features of the milder form of the human disease

• Main Authors: Heike Wolf (Author), Markus Damme (Author), Stijn Stroobants (Author), Rudi D'Hooge (Author), Hans Christian Beck (Author), Irm Hermans-Borgmeyer (Author), Renate Lüllmann-Rauch (Author), Thomas Dierks (Author), Torben Lübke (Author)
• Format: Book
• Published: The Company of Biologists, 2016-09-01T00:00:00Z.
• Subjects: article
• Online Access: Connect to this object online.

Fucosidosis is a rare lysosomal storage disorder caused by the inherited deficiency of the lysosomal hydrolase α-L-fucosidase, which leads to an impaired degradation of fucosylated glycoconjugates. Here, we report the generation of a fucosidosis mouse model, in which the gene for lysosomal α-L-fucos...