Long-term enzyme replacement therapy improves neurocognitive functioning and hippocampal synaptic plasticity in immune-tolerant alpha-mannosidosis mice

Long-term enzyme replacement therapy improves neurocognitive functioning and hippocampal synaptic plasticity in immune-tolerant alpha-mannosidosis mice

Alpha-mannosidosis is a glycoproteinosis caused by deficiency of lysosomal acid alpha-mannosidase (LAMAN), which markedly affects neurons of the central nervous system (CNS), and causes pathognomonic intellectual dysfunction in the clinical condition. Cognitive improvement consequently remains a maj...

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Bibliographic Details
Main Authors: Stijn Stroobants, Markus Damme, Ann Van der Jeugd, Ben Vermaercke, Claes Andersson, Jens Fogh, Paul Saftig, Judith Blanz, Rudi D'Hooge
Format: Article
Language:English
Published: Elsevier 2017-10-01
Series:Neurobiology of Disease
Subjects:
Enzyme replacement therapy
Lysosomal storage disorder
Alpha-mannosidosis
Synaptic plasticity
Long-term potentiation
Hippocampus
Online Access:http://www.sciencedirect.com/science/article/pii/S0969996117301663

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http://www.sciencedirect.com/science/article/pii/S0969996117301663

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