Sensory involvement in the SOD1-G93A mouse model of amyotrophic lateral sclerosis
Experimental & Molecular Medicine
; : 140-150, 2009.
Article
de En
| WPRIM
| ID: wpr-76616
Bibliothèque responsable:
WPRO
ABSTRACT
A subset of patients of amyotrophic lateral sclerosis (ALS) present with mutation of Cu/Zn superoxide dismutase 1 (SOD1), and such mutants caused an ALS-like disorder when expressed in rodents. These findings implicated SOD1 in ALS pathogenesis and made the transgenic animals a widely used ALS model. However, previous studies of these animals have focused largely on motor neuron damage. We report herein that the spinal cords of mice expressing a human SOD1 mutant (hSOD1-G93A), besides showing typical destruction of motor neurons and axons, exhibit significant damage in the sensory system, including Wallerian-like degeneration in axons of dorsal root and dorsal funiculus, and mitochondrial damage in dorsal root ganglia neurons. Thus, hSOD1-G93A mutation causes both motor and sensory neuropathies, and as such the disease developed in the transgenic mice very closely resembles human ALS.
Mots clés
Texte intégral:
1
Indice:
WPRIM
Sujet Principal:
Cellules réceptrices sensorielles
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Moelle spinale
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Axones
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Superoxide dismutase
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Souris transgéniques
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Modèles animaux de maladie humaine
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Ganglions sensitifs des nerfs spinaux
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Sclérose latérale amyotrophique
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Mitochondries
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Motoneurones
Limites du sujet:
Animals
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Humans
langue:
En
Texte intégral:
Experimental & Molecular Medicine
Année:
2009
Type:
Article