dizocilpine-maleate and Zellweger-Syndrome

dizocilpine-maleate has been researched along with Zellweger-Syndrome* in 1 studies

Other Studies

1 other study(ies) available for dizocilpine-maleate and Zellweger-Syndrome

Article	Year
Neuronal migration disorder in Zellweger mice is secondary to glutamate receptor dysfunction. Annals of neurology, 2000, Volume: 48, Issue:3 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. Topics: Animals; Autoradiography; Calcium; Cell Movement; Dizocilpine Maleate; Female; Male; Mice; Neurons; Receptors, GABA; Receptors, Glutamate; Receptors, N-Methyl-D-Aspartate; Zellweger Syndrome	2000

Article

Year

Neuronal migration disorder in Zellweger mice is secondary to glutamate receptor dysfunction.

Annals of neurology, 2000, Volume: 48, Issue:3

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.

Topics: Animals; Autoradiography; Calcium; Cell Movement; Dizocilpine Maleate; Female; Male; Mice; Neurons; Receptors, GABA; Receptors, Glutamate; Receptors, N-Methyl-D-Aspartate; Zellweger Syndrome

2000