Sensory involvement in the SOD1-G93A mouse model of amyotrophic lateral sclerosis
Experimental & Molecular Medicine
; : 140-150, 2009.
Article
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| 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
Asunto principal:
Células Receptoras Sensoriales
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Médula Espinal
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Axones
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Superóxido Dismutasa
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Ratones Transgénicos
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Modelos Animales de Enfermedad
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Ganglios Espinales
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Esclerosis Amiotrófica Lateral
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Mitocondrias
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Neuronas Motoras
Límite:
Animals
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Humans
Idioma:
En
Revista:
Experimental & Molecular Medicine
Año:
2009
Tipo del documento:
Article