Electrophysiological analysis of cultured fetal mouse dorsal root ganglion neurons transgenic for human superoxide dismutase-1, a gene in the Down syndrome region of chromosome 21.

Our recent whole cell patch-pipette studies have shown that human trisomy 21 (Down syndrome) cultured fetal dorsal root ganglion (DRG) neurons have accelerated rates of action potential depolarization and repolarization, with reduced spike duration, compared to control neurons. Similar observations were made using DRG neurons from the trisomy 16 mouse, an animal model of trisomy 21. In this study we have used transgenic mice in order to investigate the relationship between excess gene dosage and neurophysiological abnormalities. DRG neurons which possessed additional copies of the gene for human superoxide dismutase-1 (SOD), a gene from the Down syndrome region of chromosome 21, were compared to normal neurons. No electrophysiological differences were found between the two groups of neurons, indicating that increased dosage of the SOD gene alone is not causal to action potential dysfunction found in trisomy 21 and trisomy 16 neurons.