Exp Mol Med.  2009 Mar;41(3):140-150. 10.3858/emm.2009.41.3.017.

Sensory involvement in the SOD1-G93A mouse model of amyotrophic lateral sclerosis

• Guo YS
• Wu DX
• Wu HR
• Wu SY
• Yang C
• Li B
• Bu H
• Zhang YS
• Li CY

• KMID: 1123461
• DOI: http://doi.org/10.3858/emm.2009.41.3.017

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.

Keyword

amyotrophic lateral sclerosis; mutation; nerve degeneration; spinal cord; spinal nerve roots; superoxide dismutase 1

MeSH Terms

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