Phospho-adducin distribution in a murine transgenic model of amyotrophic lateral sclerosis

Amyotrophic lateral sclerosis (ALS) is a human neurodegenerative disorder characterized by the selective death of neurons in spinal cord, brain stem and cortex. The pathogenesis of ALS is still unknown. Adducins (Add) are a family of cytoskeletal associated proteins. Recent studies have shown that protein levels of phosphorylated adducin (phospho-adducin, p-Add) are increased in spinal cord tissue from ALS patients. I have evaluated the distribution of p-Add imrnunoreactivity in spinal cords of mice over-expressing mutant human superoxide dismutase (mSOD), an animal model of ALS, and in control littermates. Spinal cords of mSOD mice having motoneuron loss exhibit significantly greater p-Add immunoreactivity in ventral horn and dorsal horn, compared to control animals. The findings demonstrate increased immunoreactivity of phosphorylated adducin in a murine model of ALS. This increased p-Add immunoreactivity may be evidence for aberrant phosphorylation events contributing to motoneuron loss in mSOD mice.