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KMID : 0620920090410030140
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Experimental & Molecular Medicine
2009 Volume.41 No. 3 p.140 ~ p.150
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Sensory involvement in the SOD1-G93A mouse model of amyotrophic lateral sclerosis
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Guo Yan-Su
Zhang Yue-sheng
Li Chun-Yan
Wu Dong-Xia
Wu Hong-Ran
Wu Shu-Yu
Yang Cheng
Li Bin
Bu Hui
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Abstract
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A subset of patients of amyotrophic lateral sclerosis (ALS) present with mutation of Cu/Zn superoxide dismutase 1 (SOD1), and such mutants caused an ALSlike 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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KEYWORD
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amyotrophic lateral sclerosis, mutation, nerve degeneration, spinal cord, spinal nerve roots, superoxide dismutase 1
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