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Title: Neuronal migration disorder in Zellweger mice is secondary to glutamate receptor dysfunction
Authors: Gressens, P ×
Baes, Myriam
Leroux, P
Lombet, A
Van Veldhoven, Paul P
Janssen, Anneleen
Vamecq, J
Marret, S
Evrard, P #
Issue Date: Oct-2000
Publisher: American Neurological Association
Series Title: Annals of Neurology vol:48 issue:3 pages:336-43
Abstract: Disorders of neuronal migration in cerebral cortex are associated with neurological impairments, including mental retardation and epilepsy. Their causes and pathophysiology remain largely unknown, however. In patients with Zellweger disease, a lethal panperoxisomal disorder, and in mice lacking the Pxr1 import receptor for peroxisomal matrix proteins, the absence of peroxisomes leads to abnormal neuronal migration. Analysis of Pxr1-/- mice revealed that the migration defect was caused by altered N-methyl-D-aspartate (NMDA) glutamate receptor-mediated calcium mobilization. This NMDA receptor dysfunction was linked to a deficit in platelet-activating factor, a phenomenon related to peroxisome impairment. These findings confirm NMDA receptor involvement in neuronal migration and suggest a link between peroxisome metabolism and NMDA receptor efficacy.
ISSN: 0364-5134
Publication status: published
KU Leuven publication type: IT
Appears in Collections:Cell Metabolism
Pharmacology Section (-)
Laboratory of Lipid Biochemistry and Protein Interactions
× corresponding author
# (joint) last author

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