{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,10,24]],"date-time":"2026-10-24T01:40:06Z","timestamp":1792806006638,"version":"4.5.0"},"reference-count":46,"publisher":"Wiley","issue":"19","license":[{"start":{"date-parts":[[2020,9,4]],"date-time":"2020-09-04T00:00:00Z","timestamp":1599177600000},"content-version":"vor","delay-in-days":0,"URL":"http:\/\/onlinelibrary.wiley.com\/termsAndConditions#vor"}],"funder":[{"DOI":"10.13039\/501100004836","name":"Det Frie Forskningsr\u00e5d","doi-asserted-by":"publisher","award":["8020\u201000330"],"award-info":[{"award-number":["8020\u201000330"]}],"id":[{"id":"10.13039\/501100004836","id-type":"DOI","asserted-by":"publisher"}]}],"content-domain":{"domain":["physoc.onlinelibrary.wiley.com"],"crossmark-restriction":true},"short-container-title":["The Journal of Physiology"],"published-print":{"date-parts":[[2020,10]]},"abstract":"<jats:sec>\n                    <jats:title>Key points<\/jats:title>\n                    <jats:p>\n                      <jats:list list-type=\"bullet\">\n                        <jats:list-item>\n                          <jats:p>\n                            Although\n                            <jats:italic>in vitro<\/jats:italic>\n                            recordings using neonatal preparations from mouse models of amyotrophic lateral sclerosis (ALS) suggest increased motoneurone excitability,\n                            <jats:italic>in vivo<\/jats:italic>\n                            recordings in adult ALS mouse models have been conflicting.\n                          <\/jats:p>\n                        <\/jats:list-item>\n                        <jats:list-item>\n                          <jats:p>In adult G93A SOD1\u00a0models, spinal motoneurones have previously been shown to have deficits in repetitive firing, in contrast to the G127X SOD1\u00a0mouse model.<\/jats:p>\n                        <\/jats:list-item>\n                        <jats:list-item>\n                          <jats:p>\n                            Our\n                            <jats:italic>in vivo<\/jats:italic>\n                            intracellular recordings in barbiturate\u2010anaesthetized adult male G93A SOD1\u00a0mice reveal that the incidence of failure to fire with current injection was equally low in control and ALS mice (\u223c2%).\n                          <\/jats:p>\n                        <\/jats:list-item>\n                        <jats:list-item>\n                          <jats:p>We show that failure to fire repetitively can be a consequence of experimental protocol and should not be used alone to classify otherwise normal motoneurones as hypo\u2010excitable.<\/jats:p>\n                        <\/jats:list-item>\n                        <jats:list-item>\n                          <jats:p>Motoneurones in the G93A SOD1\u00a0mice showed an increased response to inputs, with lower rheobase, higher input\u2013output gains and increased activation of persistent inward currents.<\/jats:p>\n                        <\/jats:list-item>\n                      <\/jats:list>\n                    <\/jats:p>\n                  <\/jats:sec>\n                  <jats:sec>\n                    <jats:title>Abstract<\/jats:title>\n                    <jats:p>\n                      <jats:italic>In vitro<\/jats:italic>\n                      studies from transgenic amyotrophic lateral sclerosis models have suggested an increased excitability of spinal motoneurones. However,\n                      <jats:italic>in vivo<\/jats:italic>\n                      intracellular recordings from adult amyotrophic lateral sclerosis mice models have produced conflicting findings. Previous investigations using barbiturate anaesthetized G93A SOD1\u00a0mice have suggested that some motoneurones are hypo\u2010excitable, defined by deficits in repetitive firing. Our own previous recordings in G127X SOD1\u00a0mice using different anaesthesia, however, showed no repetitive firing deficits and increased persistent inward currents at symptom onset. These discrepancies may be a result of differences between models, symptomatic stage, anaesthesia or technical differences. To investigate this, we repeated our original experiments, but in adult male G93A SOD1\u00a0mice, at both presymptomatic and symptomatic stages, under barbiturate anaesthesia.\n                      <jats:italic>In vivo<\/jats:italic>\n                      intracellular recordings from antidromically identified spinal motoneurones revealed that the incidence of failure to fire with current injection was equally low in control and G93A SOD1\u00a0mice (\u223c2%). Motoneurones in G93A SOD1\u00a0mice fired significantly more spontaneous action potentials. Rheobase was significantly lower and the input resistance and input\u2013output gain were significantly higher in both presymptomatic and symptomatic G93A SOD1\u00a0mice. This was despite a significant increase in the duration of the post\u2010spike after\u2010hyperpolarization in both presymptomatic and symptomatic G93A SOD1\u00a0mice. Finally, evidence of increased activation of persistent inward currents was seen in both presymptomatic and symptomatic G93A SOD1\u00a0mice. Our results do not confirm previous reports of hypo\u2010excitability of spinal motoneurones in the G93A SOD1\u00a0mouse and demonstrate that the motoneurones show an increased response to inputs.\n                    <\/jats:p>\n                  <\/jats:sec>","DOI":"10.1113\/jp280097","type":"journal-article","created":{"date-parts":[[2020,7,27]],"date-time":"2020-07-27T09:58:10Z","timestamp":1595843890000},"page":"4385-4403","update-policy":"https:\/\/doi.org\/10.1002\/crossmark_policy","source":"Crossref","is-referenced-by-count":47,"title":["Spinal motoneurones are intrinsically more responsive in the adult G93A SOD1\u00a0mouse model of amyotrophic lateral sclerosis"],"prefix":"10.1113","volume":"598","author":[{"given":"Dennis B.","family":"Jensen","sequence":"first","affiliation":[{"name":"Department of Neuroscience University of Copenhagen Panum Institute  Blegdamsvej 3 Copenhagen N Denmark"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Marion","family":"Kadlecova","sequence":"additional","affiliation":[{"name":"Department of Neuroscience University of Copenhagen Panum Institute  Blegdamsvej 3 Copenhagen N Denmark"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Ilary","family":"Allodi","sequence":"additional","affiliation":[{"name":"Department of Neuroscience University of Copenhagen Panum Institute  Blegdamsvej 3 Copenhagen N Denmark"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0002-4885-4326","authenticated-orcid":false,"given":"Claire F.","family":"Meehan","sequence":"additional","affiliation":[{"name":"Department of Neuroscience University of Copenhagen Panum Institute  Blegdamsvej 3 Copenhagen N Denmark"}],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"311","published-online":{"date-parts":[[2020,9,4]]},"reference":[{"key":"e_1_2_5_2_1","doi-asserted-by":"publisher","DOI":"10.1016\/j.brainres.2011.02.060"},{"key":"e_1_2_5_3_1","doi-asserted-by":"publisher","DOI":"10.7554\/eLife.23011"},{"key":"e_1_2_5_4_1","doi-asserted-by":"publisher","DOI":"10.1152\/jn.00587.2012"},{"key":"e_1_2_5_5_1","doi-asserted-by":"publisher","DOI":"10.1016\/0028-3908(85)90196-0"},{"key":"e_1_2_5_6_1","doi-asserted-by":"publisher","DOI":"10.1056\/NEJM199403033300901"},{"key":"e_1_2_5_7_1","doi-asserted-by":"publisher","DOI":"10.1038\/s41598-019-57314-w"},{"key":"e_1_2_5_8_1","doi-asserted-by":"publisher","DOI":"10.1152\/jn.00646.2005"},{"key":"e_1_2_5_9_1","doi-asserted-by":"publisher","DOI":"10.1113\/jphysiol.2006.107292"},{"key":"e_1_2_5_10_1","doi-asserted-by":"publisher","DOI":"10.1002\/brb3.104"},{"key":"e_1_2_5_11_1","doi-asserted-by":"publisher","DOI":"10.1016\/S0028-3908(98)00081-1"},{"key":"e_1_2_5_12_1","doi-asserted-by":"publisher","DOI":"10.1016\/j.nbd.2016.04.009"},{"key":"e_1_2_5_13_1","doi-asserted-by":"publisher","DOI":"10.1016\/j.jns.2007.10.024"},{"key":"e_1_2_5_14_1","doi-asserted-by":"publisher","DOI":"10.1113\/jphysiol.2013.265843"},{"key":"e_1_2_5_15_1","doi-asserted-by":"publisher","DOI":"10.1038\/ncomms6999"},{"key":"e_1_2_5_16_1","unstructured":"DimintiyanovaKP WieneckeJ JensenDB&MeehanCF(2016).Time course of axon initial segment plasticity caudal to a complete spinal cord transection in rats.Society for Neuroscience Abstracts Program No. 142.117. 2016 Neuroscience Meetin Planer. 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