Neurophysiological abnormalities in cultured dorsal root ganglion neurons from the trisomy-16 mouse fetus, a model for Down syndrome.
The trisomy-16 mouse is considered to be a model of human trisomy-21 (Down syndrome). We have examined the electrical membrane properties of cultured dorsal root ganglion (DRG) neurons from normal and trisomy-16 fetuses. Trisomy-16 neurons had significantly accelerated rates of action potential depolarization and repolarization compared to diploid neurons, resulting in decreased spike duration. These changes match those reported in human trisomy-21 DRG neurons. Such abnormalities may contribute to the mental retardation characteristic of Down syndrome.
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Laboratory of Neurosciences, National Institute on Aging, Bethesda, MD 20892.
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Rapoport
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D000200:Action Potentials; D000818:Animals; D002478:Cells, Cultured; D004195:Disease Models, Animal; D004314:Down Syndrome / Q000503:physiopathology*; D005727:Ganglia, Spinal / Q000166:cytology / Q000503:physiopathology*; D008564:Membrane Potentials; D051379:Mice