title: Sensory involvement in the SOD1-G93A mouse model of amyotrophic lateral sclerosis.
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.
authors.affiliation: Department of Neurology, Hebei Province Key Laboratory of Neurology, Institute of Cardiocerebrovascular DiseaseThe Second Hospital of Hebei Medical UniversityShijiazhuang, Hebei 050000, China.
authors.forename: Yan Su
authors.initials: YS
authors.lastname: Guo
authors.forename: Dong Xia
authors.initials: DX
authors.lastname: Wu
authors.forename: Hong Ran
authors.initials: HR
authors.lastname: Wu
authors.forename: Shu Yu
authors.initials: SY
authors.lastname: Wu
authors.forename: Cheng
authors.initials: C
authors.lastname: Yang
authors.forename: Bin
authors.initials: B
authors.lastname: Li
authors.forename: Hui
authors.initials: H
authors.lastname: Bu
authors.forename: Yue Sheng
authors.initials: YS
authors.lastname: Zhang
authors.forename: Chun Yan
authors.initials: CY
authors.lastname: Li
mesh_terms: D000690:Amyotrophic Lateral Sclerosis / Q000201:enzymology / Q000473:pathology*; D000818:Animals; D001369:Axons / Q000473:pathology*; D004195:Disease Models, Animal; D005727:Ganglia, Spinal / Q000473:pathology; D006801:Humans; D051379:Mice; D008822:Mice, Transgenic; D008928:Mitochondria / Q000473:pathology; D009046:Motor Neurons / Q000378:metabolism / Q000473:pathology; D009154:Mutation; D009410:Nerve Degeneration / Q000473:pathology*; D011984:Sensory Receptor Cells / Q000473:pathology*; D013116:Spinal Cord / Q000473:pathology*; D013482:Superoxide Dismutase / Q000235:genetics / Q000502:physiology*; D000072105:Superoxide Dismutase-1
chemical_list: C000606290:SOD1 protein, human; C000606291:Sod1 protein, mouse; D013482:Superoxide Dismutase; D000072105:Superoxide Dismutase-1