{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,10,6]],"date-time":"2026-10-06T13:26:09Z","timestamp":1791293169585,"version":"4.1.0"},"reference-count":49,"publisher":"Wiley","issue":"5","license":[{"start":{"date-parts":[[2014,2,7]],"date-time":"2014-02-07T00:00:00Z","timestamp":1391731200000},"content-version":"vor","delay-in-days":0,"URL":"http:\/\/onlinelibrary.wiley.com\/termsAndConditions#vor"}],"content-domain":{"domain":[],"crossmark-restriction":false},"short-container-title":["CNS Neurosci Ther"],"published-print":{"date-parts":[[2014,5]]},"abstract":"<jats:title>Summary<\/jats:title><jats:sec><jats:title>Aims<\/jats:title><jats:p>Me<jats:styled-content style=\"fixed-case\">CP<\/jats:styled-content>2 gene mutations are associated with Rett syndrome and X\u2010linked mental retardation (<jats:styled-content style=\"fixed-case\">XLMR<\/jats:styled-content>), diseases characterized by abnormal brain development and function. Recently, we created a novel Me<jats:styled-content style=\"fixed-case\">CP<\/jats:styled-content>2 A140V mutation mouse model that exhibited abnormalities of cell packing density and dendritic branching consistent with that seen in Rett syndrome patients as well as other Me<jats:styled-content style=\"fixed-case\">CP<\/jats:styled-content>2 mutant mouse models. Therefore, we hypothesized that some deficits of neuronal and synaptic functions might also be present in the A140V mutant model.<\/jats:p><\/jats:sec><jats:sec><jats:title>Methods<\/jats:title><jats:p>Here, we tested our hypothesis in hippocampal slices using electrophysiological recordings.<\/jats:p><\/jats:sec><jats:sec><jats:title>Results<\/jats:title><jats:p>We found that in young A140V mutant mice (3\u2010 to 4\u2010week\u2010old), hippocampal <jats:styled-content style=\"fixed-case\">CA<\/jats:styled-content>1 pyramidal neurons exhibited more positive resting membrane potential, increased action potential (<jats:styled-content style=\"fixed-case\">AP<\/jats:styled-content>) firing frequency induced by injection of depolarizing current, wider <jats:styled-content style=\"fixed-case\">AP<\/jats:styled-content> duration, and smaller after hyperpolarization potential compared to neurons prepared from age\u2010matched wild\u2010type mice, suggesting a neuronal hyperexcitation. At the synaptic level, A140V mutant neurons exhibited a reduced frequency of spontaneous <jats:styled-content style=\"fixed-case\">IPSC<\/jats:styled-content>s (inhibitory postsynaptic potentials) and an enhanced probability of evoked glutamate release, both suggesting neuronal hyperexcitation. However, hippocampal <jats:styled-content style=\"fixed-case\">CA<\/jats:styled-content>1 long\u2010term potentiation was not significantly different between A140V and <jats:styled-content style=\"fixed-case\">WT<\/jats:styled-content> mice. In adult mice (11\u2010 to 13\u2010month\u2010old), in addition to neuronal hyperexcitation, we also found significant deficits of both short\u2010term and long\u2010term potentiation of <jats:styled-content style=\"fixed-case\">CA<\/jats:styled-content>3\u2010<jats:styled-content style=\"fixed-case\">CA<\/jats:styled-content>1 synapses in A140V mice compared to <jats:styled-content style=\"fixed-case\">WT<\/jats:styled-content> mice.<\/jats:p><\/jats:sec><jats:sec><jats:title>Conclusions<\/jats:title><jats:p>These results clearly illustrate the age\u2010dependent abnormalities of neuronal and synaptic function in the Me<jats:styled-content style=\"fixed-case\">CP<\/jats:styled-content>2 A140V mutant mouse model, which provides new insights into the understanding of the pathogenesis of Rett syndrome.<\/jats:p><\/jats:sec>","DOI":"10.1111\/cns.12229","type":"journal-article","created":{"date-parts":[[2014,2,7]],"date-time":"2014-02-07T09:28:42Z","timestamp":1391765322000},"page":"420-428","source":"Crossref","is-referenced-by-count":15,"title":["Electrophysiological Phenotypes of Me<scp>CP<\/scp>2 A140V Mutant Mouse Model"],"prefix":"10.1111","volume":"20","author":[{"given":"Lu\u2010Yao","family":"Ma","sequence":"first","affiliation":[{"name":"Division of Neurology Barrow Neurological Institute St. Joseph's Hospital and Medical Center Phoenix AZ USA"},{"name":"Department of Cardiothoracic Surgery The First Affiliated Hospital with Nanjing Medical University Nanjing China"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Chen","family":"Wu","sequence":"additional","affiliation":[{"name":"Division of Neurology Barrow Neurological Institute St. Joseph's Hospital and Medical Center Phoenix AZ USA"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Yu","family":"Jin","sequence":"additional","affiliation":[{"name":"Division of Neurology Barrow Neurological Institute St. Joseph's Hospital and Medical Center Phoenix AZ USA"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Ming","family":"Gao","sequence":"additional","affiliation":[{"name":"Division of Neurology Barrow Neurological Institute St. Joseph's Hospital and Medical Center Phoenix AZ USA"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Guo\u2010Hui","family":"Li","sequence":"additional","affiliation":[{"name":"Division of Neurology Barrow Neurological Institute St. Joseph's Hospital and Medical Center Phoenix AZ USA"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Dharshaun","family":"Turner","sequence":"additional","affiliation":[{"name":"Division of Neurology Barrow Neurological Institute St. Joseph's Hospital and Medical Center Phoenix AZ USA"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Jian\u2010Xin","family":"Shen","sequence":"additional","affiliation":[{"name":"Department of Physiology Shantou University Medical College Shantou China"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Shi\u2010Jiang","family":"Zhang","sequence":"additional","affiliation":[{"name":"Department of Cardiothoracic Surgery The First Affiliated Hospital with Nanjing Medical University Nanjing China"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Vinodh","family":"Narayanan","sequence":"additional","affiliation":[{"name":"Pediatric Neurology Department Barrow Neurological Institute St. Joseph's Hospital and Medical Center Phoenix AZ USA"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Garilyn","family":"Jentarra","sequence":"additional","affiliation":[{"name":"Pediatric Neurology Department Barrow Neurological Institute St. Joseph's Hospital and Medical Center Phoenix AZ USA"},{"name":"Department of Biochemistry Midwestern University Glendale AZ USA"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Jie","family":"Wu","sequence":"additional","affiliation":[{"name":"Division of Neurology Barrow Neurological Institute St. Joseph's Hospital and Medical Center Phoenix AZ USA"},{"name":"Department of Physiology Shantou University Medical College Shantou China"}],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"311","published-online":{"date-parts":[[2014,2,7]]},"reference":[{"key":"e_1_2_8_2_1","doi-asserted-by":"publisher","DOI":"10.1042\/BST0360575"},{"key":"e_1_2_8_3_1","doi-asserted-by":"publisher","DOI":"10.1126\/science.1153252"},{"key":"e_1_2_8_4_1","doi-asserted-by":"publisher","DOI":"10.1038\/nrg1878"},{"key":"e_1_2_8_5_1","doi-asserted-by":"publisher","DOI":"10.1016\/j.gde.2007.02.003"},{"key":"e_1_2_8_6_1","doi-asserted-by":"publisher","DOI":"10.1074\/jbc.M305308200"},{"key":"e_1_2_8_7_1","doi-asserted-by":"publisher","DOI":"10.1016\/S0092-8674(00)81887-5"},{"key":"e_1_2_8_8_1","doi-asserted-by":"publisher","DOI":"10.1016\/j.molcel.2010.01.030"},{"key":"e_1_2_8_9_1","doi-asserted-by":"publisher","DOI":"10.1038\/13810"},{"key":"e_1_2_8_10_1","doi-asserted-by":"publisher","DOI":"10.1016\/j.neuron.2007.10.001"},{"key":"e_1_2_8_11_1","doi-asserted-by":"publisher","DOI":"10.1111\/j.1651-2227.1985.tb10993.x"},{"key":"e_1_2_8_12_1","doi-asserted-by":"publisher","DOI":"10.1002\/ana.410140412"},{"key":"e_1_2_8_13_1","doi-asserted-by":"publisher","DOI":"10.1002\/ana.22124"},{"key":"e_1_2_8_14_1","doi-asserted-by":"publisher","DOI":"10.1177\/1073858403260995"},{"key":"e_1_2_8_15_1","doi-asserted-by":"publisher","DOI":"10.1212\/01.wnl.0000223318.28938.45"},{"key":"e_1_2_8_16_1","doi-asserted-by":"publisher","DOI":"10.1097\/00005072-199503000-00006"},{"key":"e_1_2_8_17_1","doi-asserted-by":"publisher","DOI":"10.1212\/WNL.45.8.1581"},{"key":"e_1_2_8_18_1","doi-asserted-by":"publisher","DOI":"10.1097\/00001756-199407000-00025"},{"key":"e_1_2_8_19_1","doi-asserted-by":"publisher","DOI":"10.1016\/j.nbd.2009.05.001"},{"key":"e_1_2_8_20_1","doi-asserted-by":"publisher","DOI":"10.1002\/ajmg.a.33184"},{"key":"e_1_2_8_21_1","doi-asserted-by":"publisher","DOI":"10.1016\/S1090-3798(02)00134-4"},{"key":"e_1_2_8_22_1","doi-asserted-by":"publisher","DOI":"10.1212\/WNL.58.2.226"},{"key":"e_1_2_8_23_1","doi-asserted-by":"publisher","DOI":"10.1086\/339553"},{"key":"e_1_2_8_24_1","doi-asserted-by":"publisher","DOI":"10.1136\/jmg.39.2.132"},{"key":"e_1_2_8_25_1","doi-asserted-by":"publisher","DOI":"10.1016\/S0014-5793(00)01994-3"},{"key":"e_1_2_8_26_1","doi-asserted-by":"publisher","DOI":"10.1002\/humu.10130"},{"key":"e_1_2_8_27_1","doi-asserted-by":"publisher","DOI":"10.1186\/1471-2202-11-19"},{"key":"e_1_2_8_28_1","doi-asserted-by":"publisher","DOI":"10.1073\/pnas.0506071102"},{"key":"e_1_2_8_29_1","doi-asserted-by":"publisher","DOI":"10.1523\/JNEUROSCI.1019-09.2009"},{"key":"e_1_2_8_30_1","doi-asserted-by":"publisher","DOI":"10.1016\/j.biopsych.2009.12.022"},{"key":"e_1_2_8_31_1","doi-asserted-by":"publisher","DOI":"10.1523\/JNEUROSCI.2623-05.2006"},{"key":"e_1_2_8_32_1","doi-asserted-by":"publisher","DOI":"10.1016\/j.nbd.2005.07.005"},{"key":"e_1_2_8_33_1","doi-asserted-by":"publisher","DOI":"10.1016\/j.neuron.2007.08.018"},{"key":"e_1_2_8_34_1","doi-asserted-by":"publisher","DOI":"10.1002\/hipo.20389"},{"key":"e_1_2_8_35_1","doi-asserted-by":"publisher","DOI":"10.1152\/jn.00601.2009"},{"key":"e_1_2_8_36_1","doi-asserted-by":"publisher","DOI":"10.1152\/jn.00826.2007"},{"key":"e_1_2_8_37_1","doi-asserted-by":"publisher","DOI":"10.1523\/JNEUROSCI.3156-09.2009"},{"key":"e_1_2_8_38_1","doi-asserted-by":"publisher","DOI":"10.1016\/j.jneumeth.2006.06.006"},{"key":"e_1_2_8_39_1","doi-asserted-by":"publisher","DOI":"10.1152\/jn.2000.84.3.1355"},{"key":"e_1_2_8_40_1","doi-asserted-by":"publisher","DOI":"10.1016\/j.neuropharm.2005.01.003"},{"key":"e_1_2_8_41_1","doi-asserted-by":"publisher","DOI":"10.1152\/jn.01318.2005"},{"key":"e_1_2_8_42_1","doi-asserted-by":"publisher","DOI":"10.1038\/nn1687"},{"key":"e_1_2_8_43_1","doi-asserted-by":"publisher","DOI":"10.1523\/JNEUROSCI.23-35-11142.2003"},{"key":"e_1_2_8_44_1","doi-asserted-by":"publisher","DOI":"10.1038\/nn1058"},{"key":"e_1_2_8_45_1","doi-asserted-by":"publisher","DOI":"10.1016\/S0092-8674(00)81509-3"},{"key":"e_1_2_8_46_1","doi-asserted-by":"publisher","DOI":"10.1038\/301603a0"},{"key":"e_1_2_8_47_1","doi-asserted-by":"publisher","DOI":"10.1523\/JNEUROSCI.1943-10.2010"},{"key":"e_1_2_8_48_1","doi-asserted-by":"publisher","DOI":"10.1146\/annurev.physiol.64.092501.114547"},{"key":"e_1_2_8_49_1","doi-asserted-by":"publisher","DOI":"10.1038\/nature09582"},{"key":"e_1_2_8_50_1","doi-asserted-by":"publisher","DOI":"10.1152\/jn.00800.2010"}],"container-title":["CNS Neuroscience &amp; Therapeutics"],"original-title":[],"language":"en","link":[{"URL":"https:\/\/api.wiley.com\/onlinelibrary\/tdm\/v1\/articles\/10.1111%2Fcns.12229","content-type":"unspecified","content-version":"vor","intended-application":"text-mining"},{"URL":"https:\/\/onlinelibrary.wiley.com\/doi\/pdf\/10.1111\/cns.12229","content-type":"unspecified","content-version":"vor","intended-application":"similarity-checking"}],"deposited":{"date-parts":[[2023,9,24]],"date-time":"2023-09-24T14:01:50Z","timestamp":1695564110000},"score":1,"resource":{"primary":{"URL":"https:\/\/onlinelibrary.wiley.com\/doi\/10.1111\/cns.12229"}},"subtitle":[],"short-title":[],"issued":{"date-parts":[[2014,2,7]]},"references-count":49,"journal-issue":{"issue":"5","published-print":{"date-parts":[[2014,5]]}},"alternative-id":["10.1111\/cns.12229"],"URL":"https:\/\/doi.org\/10.1111\/cns.12229","archive":["Portico"],"relation":{},"ISSN":["1755-5930","1755-5949"],"issn-type":[{"value":"1755-5930","type":"print"},{"value":"1755-5949","type":"electronic"}],"subject":[],"published":{"date-parts":[[2014,2,7]]}}}