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

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 p...

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Autores principales:	Hongmei Zhang, Natallia Robinson, Chiayen Wu, Wenlan Wang, Melissa A Harrington
Formato:	article
Lenguaje:	EN
Publicado:	Public Library of Science (PLoS) 2010
Materias:	Medicine R Science Q
Acceso en línea:	https://doaj.org/article/34e4ec0cd8664a8abdb37c4746ee51a6
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spelling	oai:doaj.org-article:34e4ec0cd8664a8abdb37c4746ee51a62021-12-02T20:19:56ZElectrophysiological properties of motor neurons in a mouse model of severe spinal muscular atrophy: in vitro versus in vivo development.1932-620310.1371/journal.pone.0011696https://doaj.org/article/34e4ec0cd8664a8abdb37c4746ee51a62010-07-01T00:00:00Zhttps://www.ncbi.nlm.nih.gov/pmc/articles/pmid/20657731/?tool=EBIhttps://doaj.org/toc/1932-6203We 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 survival between cells from SMA embryos and their unaffected littermates. Our results represent the first report on the electrophysiology of SMN-deficient motor neurons, and suggest that motor neuron development in vitro follows a different path than in vivo development, a path in which loss of SMN expression has little effect on motor neuron function and survival.Hongmei ZhangNatallia RobinsonChiayen WuWenlan WangMelissa A HarringtonPublic Library of Science (PLoS)articleMedicineRScienceQENPLoS ONE, Vol 5, Iss 7, p e11696 (2010)
institution	DOAJ
collection	DOAJ
language	EN
topic	Medicine R Science Q
spellingShingle	Medicine R Science Q Hongmei Zhang Natallia Robinson Chiayen Wu Wenlan Wang Melissa A Harrington Electrophysiological properties of motor neurons in a mouse model of severe spinal muscular atrophy: in vitro versus in vivo development.
description	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 survival between cells from SMA embryos and their unaffected littermates. Our results represent the first report on the electrophysiology of SMN-deficient motor neurons, and suggest that motor neuron development in vitro follows a different path than in vivo development, a path in which loss of SMN expression has little effect on motor neuron function and survival.
format	article
author	Hongmei Zhang Natallia Robinson Chiayen Wu Wenlan Wang Melissa A Harrington
author_facet	Hongmei Zhang Natallia Robinson Chiayen Wu Wenlan Wang Melissa A Harrington
author_sort	Hongmei Zhang
title	Electrophysiological properties of motor neurons in a mouse model of severe spinal muscular atrophy: in vitro versus in vivo development.
title_short	Electrophysiological properties of motor neurons in a mouse model of severe spinal muscular atrophy: in vitro versus in vivo development.
title_full	Electrophysiological properties of motor neurons in a mouse model of severe spinal muscular atrophy: in vitro versus in vivo development.
title_fullStr	Electrophysiological properties of motor neurons in a mouse model of severe spinal muscular atrophy: in vitro versus in vivo development.
title_full_unstemmed	Electrophysiological properties of motor neurons in a mouse model of severe spinal muscular atrophy: in vitro versus in vivo development.
title_sort	electrophysiological properties of motor neurons in a mouse model of severe spinal muscular atrophy: in vitro versus in vivo development.
publisher	Public Library of Science (PLoS)
publishDate	2010
url	https://doaj.org/article/34e4ec0cd8664a8abdb37c4746ee51a6
work_keys_str_mv	AT hongmeizhang electrophysiologicalpropertiesofmotorneuronsinamousemodelofseverespinalmuscularatrophyinvitroversusinvivodevelopment AT natalliarobinson electrophysiologicalpropertiesofmotorneuronsinamousemodelofseverespinalmuscularatrophyinvitroversusinvivodevelopment AT chiayenwu electrophysiologicalpropertiesofmotorneuronsinamousemodelofseverespinalmuscularatrophyinvitroversusinvivodevelopment AT wenlanwang electrophysiologicalpropertiesofmotorneuronsinamousemodelofseverespinalmuscularatrophyinvitroversusinvivodevelopment AT melissaaharrington electrophysiologicalpropertiesofmotorneuronsinamousemodelofseverespinalmuscularatrophyinvitroversusinvivodevelopment
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Electrophysiological properties of motor neurons in a mouse model of severe spinal muscular atrophy: in vitro versus in vivo development.

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