Early gene expression profiling of motor neurons in the SOD1G93A mouse model of amyotrophic lateral sclerosis: insights into earliest pathogenesis

Abstract

Although amyotrophic lateral sclerosis (ALS) typically presents in mid to late life, there is increasing evidence in patients and mouse models for a protracted preclinical period of motor neuron vulnerability and damage before clinical onset. We hypothesized that the seeds for the development of ALS may be sown shortly after conception and motor neuron vulnerability is induced in the perinatal period of life, leading to subsequent neuronal dysfunction and degeneration. The goal of this study was to identify the earliest gene expression patterns in the vulnerable lower motor neurons at very early and key developmental ages in the superoxide dismutase 1 (SOD1)G93A mouse model of ALS. We have i..