Sensory involvement in the SOD1-G93A mouse model of amyotrophic lateral sclerosis.
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.
Guo
Yan Su
YS
Department of Neurology, Hebei Province Key Laboratory of Neurology, Institute of Cardiocerebrovascular DiseaseThe Second Hospital of Hebei Medical UniversityShijiazhuang, Hebei 050000, China.
Wu
Dong Xia
DX
Wu
Hong Ran
HR
Wu
Shu Yu
SY
Yang
Cheng
C
Li
Bin
B
Bu
Hui
H
Zhang
Yue Sheng
YS
Li
Chun Yan
CY
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
C000606290:SOD1 protein, human; C000606291:Sod1 protein, mouse; D013482:Superoxide Dismutase; D000072105:Superoxide Dismutase-1