Electrophysiological properties of motor neurons in a mouse model of severe spinal muscular atrophy: in vitro versus in vivo development.

PloS One
Hongmei ZhangMelissa A Harrington

Abstract

We examined the electrophysiological activity of motor neurons from the mouse model of severe spinal muscular atrophy (SMA) using two different methods: whole cell patch clamp of neurons cultured from day 13 embryos; and multi-electrode recording of ventral horns in spinal cord slices from pups on post-natal days 5 and 6. We used the MED64 multi-electrode array to record electrophysiological activity from motor neurons in slices from the lumbar spinal cord of SMA pups and their unaffected littermates. Recording simultaneously from up to 32 sites across the ventral horn, we observed a significant decrease in the number of active neurons in 5-6 day-old SMA pups compared to littermates. Ventral horn activity in control pups is significantly activated by serotonin and depressed by GABA, while these agents had much less effect on SMA slices. In contrast to the large differences observed in spinal cord, neurons cultured from SMA embryos for up to 21 days showed no significant differences in electrophysiological activity compared to littermates. No differences were observed in membrane potential, frequency of spiking and synaptic activity in cells from SMA embryos compared to controls. In addition, we observed no difference in cell su...Continue Reading

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Citations

Jun 29, 2011·Disease Models & Mechanisms·James N SleighKevin Talbot
Sep 22, 2011·Neuroreport·Hongmei ZhangMelissa A Harrington
Jun 29, 2011·Brain Research·Robert M Brownstone, Douglas G Stuart
Aug 24, 2019·Frontiers in Neuroinformatics·Raghu Sesha IyengarMohan Raghavan

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Methods Mentioned

BETA
transgenic
SMA
genotyping
PCR

Software Mentioned

Matlab

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