Sensory involvement in the SOD1-G93A mouse model of amyotrophic lateral sclerosis.
10.3858/emm.2009.41.3.017
- Author:
Yan Su GUO
1
;
Dong Xia WU
;
Hong Ran WU
;
Shu Yu WU
;
Cheng YANG
;
Bin LI
;
Hui BU
;
Yue sheng ZHANG
;
Chun Yan LI
Author Information
1. Department of Neurology, The Second Hospital of Hebei Medical University Shijiazhuang, Hebei 050000, China. chunyanli5@yahoo.com.cn
- Publication Type:Original Article
- Keywords:
amyotrophic lateral sclerosis;
mutation;
nerve degeneration;
spinal cord;
spinal nerve roots;
superoxide dismutase 1
- MeSH:
Amyotrophic Lateral Sclerosis/enzymology/*pathology;
Animals;
Axons/*pathology;
Disease Models, Animal;
Ganglia, Spinal/pathology;
Humans;
Mice;
Mice, Transgenic;
Mitochondria/pathology;
Motor Neurons/metabolism/pathology;
Mutation;
Nerve Degeneration/*pathology;
Sensory Receptor Cells/*pathology;
Spinal Cord/*pathology;
Superoxide Dismutase/genetics/*physiology
- From:Experimental & Molecular Medicine
2009;41(3):140-150
- CountryRepublic of Korea
- Language:English
-
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.
