Late-Onset Motoneuron Disease Caused by a Functionally Modified AMPA Receptor Subunit

Amyotrophic lateral sclerosis (ALS) is a devastating disorder of the central nervous system in middle and old age that leads to progressive loss of spinal motoneurons. Transgenic mice overexpressing mutated human Cu2+/ Zn2+superoxide dismutase 1 (SOD1) reproduce clinical features of the familial for...

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Veröffentlicht in:Proceedings of the National Academy of Sciences - PNAS 2005-04, Vol.102 (16), p.5826-5831
Hauptverfasser: Kuner, Rohini, Groom, Anthony J., Bresink, Iris, Kornau, Hans-Christian, Stefovska, Vanya, Müller, Gerald, Hartmann, Bettina, Tschauner, Karsten, Waibel, Stefan, Ludolph, Albert C., Ikonomidou, Chrysanthy, Seeburg, Peter H., Turski, Lechoslaw, Lindauer, Martin
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container_issue 16
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container_title Proceedings of the National Academy of Sciences - PNAS
container_volume 102
creator Kuner, Rohini
Groom, Anthony J.
Bresink, Iris
Kornau, Hans-Christian
Stefovska, Vanya
Müller, Gerald
Hartmann, Bettina
Tschauner, Karsten
Waibel, Stefan
Ludolph, Albert C.
Ikonomidou, Chrysanthy
Seeburg, Peter H.
Turski, Lechoslaw
Lindauer, Martin
description Amyotrophic lateral sclerosis (ALS) is a devastating disorder of the central nervous system in middle and old age that leads to progressive loss of spinal motoneurons. Transgenic mice overexpressing mutated human Cu2+/ Zn2+superoxide dismutase 1 (SOD1) reproduce clinical features of the familial form of ALS. However, changes in SOD1 activity do not correlate with severity of motor decline in sporadic cases, indicating that targets unrelated to superoxide metabolism contribute to the pathogenesis of the disease. We show here that transgenic expression in mice of GluR-B(N)-containing L-α-amino-3-hydroxy-5-methylisoxazole-4-proprionate (AMPA) receptors with increased Ca2+permeability leads to late-onset degeneration of neurons in the spinal cord and decline of motor functions. Neuronal death progresses over the entire lifespan but manifests clinically in late adulthood, resembling the course of a slow neurodegenerative disorder. Additional transgenic expression of mutated human SOD1 accelerates disease progression, aggravates the severity of motor decline, and decreases survival. These observations link persistently elevated Ca2+influx through AMPA channels with progressive motor decline and late-onset degeneration of spinal motoneurons, indicating that functionally altered AMPA channels may be causally related to pathogenesis of sporadic ALS in humans.
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subjects AMPA receptors
Amyotrophic lateral sclerosis
Amyotrophic Lateral Sclerosis - etiology
Amyotrophic Lateral Sclerosis - metabolism
Amyotrophic Lateral Sclerosis - pathology
Animals
Biological Sciences
Brain
Brain - cytology
Brain - pathology
Calcium - metabolism
Cobalt - metabolism
Electromyography
Gene expression
Humans
In Situ Hybridization
Messenger RNA
Mice
Mice, Inbred BALB C
Mice, Transgenic
Motor ability
Motor Activity - physiology
Mutation
Nervous system
Nervous system diseases
Neurons
Neurons - metabolism
Neurons - pathology
Neurons - ultrastructure
Protein Subunits - genetics
Protein Subunits - metabolism
Receptors, AMPA - genetics
Receptors, AMPA - metabolism
Reflex - physiology
Reflexes
Rodents
Spinal cord
Spinal Cord - cytology
Spinal Cord - pathology
Superoxide Dismutase - genetics
Superoxide Dismutase - metabolism
Superoxide Dismutase-1
Transgenic animals
title Late-Onset Motoneuron Disease Caused by a Functionally Modified AMPA Receptor Subunit
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