Sensory involvement in the SOD1-G93A mouse model of amyotrophic lateral sclerosis
Experimental & Molecular Medicine
; : 140-150, 2009.
Article
em En
| WPRIM
| ID: wpr-76616
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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.
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Índice:
WPRIM
Assunto principal:
Células Receptoras Sensoriais
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Medula Espinal
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Axônios
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Superóxido Dismutase
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Camundongos Transgênicos
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Modelos Animais de Doenças
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Gânglios Espinais
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Esclerose Lateral Amiotrófica
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Mitocôndrias
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Neurônios Motores
Limite:
Animals
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Humans
Idioma:
En
Revista:
Experimental & Molecular Medicine
Ano de publicação:
2009
Tipo de documento:
Article