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.

Investigation on neurofilament in immune-mediated spinal cord motor neuron injury / 中华神经科杂志

Objective To explore the relationship between immune and pathogenesis of amyotrophic lateral sclerosis (ALS) in the investigation of neurofilaments phosphorylation and ultrastructure features in spinal cord ventral horn motor neuron injury mediated by immune.Methods Using transmission electron microscope,we studied the uhrastructure features of abnormal accumulations of neurofilaments (NF) in motoneuron of the spinal cord ventral horn,and immunohistochemically investigated neurofilaments phosphorylation.Results Electron microscope found that there was abnormal accumulation of interwoven NFs in motor neuronal perikarya and proximal axons.Immunohistochemical study revealed that the SMI-32 immunoreactive positive neurons (12.00?1.05),compared with control (18.00?1.83),were reduced (P