{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,10,22]],"date-time":"2026-10-22T12:55:11Z","timestamp":1792673711858,"version":"4.5.0"},"reference-count":18,"publisher":"Wiley","issue":"4","license":[{"start":{"date-parts":[[2017,9,23]],"date-time":"2017-09-23T00:00:00Z","timestamp":1506124800000},"content-version":"vor","delay-in-days":0,"URL":"http:\/\/onlinelibrary.wiley.com\/termsAndConditions#vor"}],"funder":[{"DOI":"10.13039\/501100000765","name":"University College London","doi-asserted-by":"publisher","id":[{"id":"10.13039\/501100000765","id-type":"DOI","asserted-by":"publisher"}]},{"DOI":"10.13039\/100000002","name":"National Institutes of Health","doi-asserted-by":"publisher","award":["5 U54 NS059065\u201005S2[NINDS\/ORD]"],"award-info":[{"award-number":["5 U54 NS059065\u201005S2[NINDS\/ORD]"]}],"id":[{"id":"10.13039\/100000002","id-type":"DOI","asserted-by":"publisher"}]},{"DOI":"10.13039\/100000002","name":"National Institutes of Health","doi-asserted-by":"publisher","award":["R13 NS057995"],"award-info":[{"award-number":["R13 NS057995"]}],"id":[{"id":"10.13039\/100000002","id-type":"DOI","asserted-by":"publisher"}]},{"DOI":"10.13039\/501100000265","name":"Medical Research Council","doi-asserted-by":"publisher","award":["G0601943"],"award-info":[{"award-number":["G0601943"]}],"id":[{"id":"10.13039\/501100000265","id-type":"DOI","asserted-by":"publisher"}]},{"DOI":"10.13039\/100000097","name":"National Center for Research Resources","doi-asserted-by":"publisher","award":["5U54 RR019498\u201005"],"award-info":[{"award-number":["5U54 RR019498\u201005"]}],"id":[{"id":"10.13039\/100000097","id-type":"DOI","asserted-by":"publisher"}]}],"content-domain":{"domain":[],"crossmark-restriction":false},"short-container-title":["Muscle and Nerve"],"published-print":{"date-parts":[[2018,4]]},"abstract":"<jats:title>ABSTRACT<\/jats:title><jats:sec><jats:title>Introduction<\/jats:title><jats:p>The gain\u2010of\u2010function mutations that underlie sodium channel myotonia (SCM) and paramyotonia congenital (PMC) produce differing clinical phenotypes. We used muscle velocity recovery cycles (MVRCs) to investigate membrane properties.<\/jats:p><\/jats:sec><jats:sec><jats:title>Methods<\/jats:title><jats:p>MVRCs and responses to trains of stimuli were compared in patients with SCM (<jats:italic>n<\/jats:italic>\u2009=\u20099), PMC (<jats:italic>n<\/jats:italic>\u2009=\u20098), and normal controls (<jats:italic>n<\/jats:italic>\u2009=\u200926).<\/jats:p><\/jats:sec><jats:sec><jats:title>Results<\/jats:title><jats:p>The muscle relative refractory period was reduced in SCM, consistent with faster recovery of the mutant sodium channels from inactivation. Both SCM and PMC showed an increased early supernormality and increased mean supernormality following multiple conditioning stimuli, consistent with slowed sodium channel inactivation. Trains of fast impulses caused a loss of amplitude in PMC, after which only half of the muscle fibers recovered, suggesting that the remainder stayed depolarized by persistent sodium currents.<\/jats:p><\/jats:sec><jats:sec><jats:title>Discussion<\/jats:title><jats:p>The differing effects of mutations on sodium channel function can be demonstrated in human subjects <jats:italic>in vivo<\/jats:italic> using this technique. <jats:italic>Muscle Nerve<\/jats:italic> <jats:bold>57<\/jats:bold>: 586\u2013594, 2018<\/jats:p><\/jats:sec>","DOI":"10.1002\/mus.25956","type":"journal-article","created":{"date-parts":[[2017,9,6]],"date-time":"2017-09-06T21:54:07Z","timestamp":1504734847000},"page":"586-594","source":"Crossref","is-referenced-by-count":25,"title":["<i>In vivo<\/i> assessment of muscle membrane properties in the sodium channel myotonias"],"prefix":"10.1002","volume":"57","author":[{"given":"S. Veronica","family":"Tan","sequence":"first","affiliation":[{"name":"MRC Centre for Neuromuscular Diseases, UCL Institute of Neurology, The National Hospital for Neurology and Neurosurgery Queen Square London WC1N 3BG United Kingdom"},{"name":"Institute of Neurology University College London Queen Square London United Kingdom"},{"name":"Department of Neurology and Neurophysiology St Thomas' Hospital, Guy's and St Thomas' NHS Foundation Trust and Department of Academic Neurosciences, Kings College London  United Kingdom"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Werner J.","family":"Z'Graggen","sequence":"additional","affiliation":[{"name":"Departments of Neurosurgery and Neurology, Inselspital Bern University Hospital, University of Bern Bern Switzerland"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Michael G.","family":"Hanna","sequence":"additional","affiliation":[{"name":"MRC Centre for Neuromuscular Diseases, UCL Institute of Neurology, The National Hospital for Neurology and Neurosurgery Queen Square London WC1N 3BG United Kingdom"},{"name":"Institute of Neurology University College London Queen Square London United Kingdom"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Hugh","family":"Bostock","sequence":"additional","affiliation":[{"name":"MRC Centre for Neuromuscular Diseases, UCL Institute of Neurology, The National Hospital for Neurology and Neurosurgery Queen Square London WC1N 3BG United Kingdom"},{"name":"Institute of Neurology University College London Queen Square London United Kingdom"},{"name":"Departments of Neurosurgery and Neurology, Inselspital Bern University Hospital, University of Bern Bern Switzerland"}],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"311","published-online":{"date-parts":[[2017,9,23]]},"reference":[{"key":"e_1_2_6_2_1","doi-asserted-by":"publisher","DOI":"10.1146\/annurev.neuro.29.051605.112815"},{"key":"e_1_2_6_3_1","doi-asserted-by":"publisher","DOI":"10.1111\/j..2003.t01-1-00691.x"},{"key":"e_1_2_6_4_1","doi-asserted-by":"publisher","DOI":"10.1002\/mus.21192"},{"key":"e_1_2_6_5_1","doi-asserted-by":"publisher","DOI":"10.1016\/j.clinph.2011.04.010"},{"key":"e_1_2_6_6_1","doi-asserted-by":"publisher","DOI":"10.1016\/j.clinph.2012.04.006"},{"key":"e_1_2_6_7_1","doi-asserted-by":"publisher","DOI":"10.1016\/j.clinph.2010.01.024"},{"key":"e_1_2_6_8_1","doi-asserted-by":"publisher","DOI":"10.1016\/j.clinph.2010.09.024"},{"key":"e_1_2_6_9_1","doi-asserted-by":"publisher","DOI":"10.1002\/mus.23293"},{"key":"e_1_2_6_10_1","doi-asserted-by":"publisher","DOI":"10.1002\/mus.24069"},{"issue":"2","key":"e_1_2_6_11_1","first-page":"249","volume":"54","author":"Tan SV","year":"2016","journal-title":"assessment of muscle membrane properties in myotonic dystrophy. Muscle Nerve"},{"key":"e_1_2_6_12_1","doi-asserted-by":"publisher","DOI":"10.1002\/mus.23267"},{"key":"e_1_2_6_13_1","doi-asserted-by":"publisher","DOI":"10.1002\/ana.22238"},{"key":"e_1_2_6_14_1","doi-asserted-by":"publisher","DOI":"10.1002\/ana.20241"},{"key":"e_1_2_6_15_1","doi-asserted-by":"publisher","DOI":"10.1113\/jphysiol.1993.sp019843"},{"key":"e_1_2_6_16_1","doi-asserted-by":"publisher","DOI":"10.1113\/jphysiol.1995.sp020864"},{"key":"e_1_2_6_17_1","doi-asserted-by":"publisher","DOI":"10.1085\/jgp.107.5.559"},{"key":"e_1_2_6_18_1","doi-asserted-by":"publisher","DOI":"10.1113\/jphysiol.1994.sp020260"},{"key":"e_1_2_6_19_1","doi-asserted-by":"publisher","DOI":"10.1073\/pnas.91.26.12785"}],"container-title":["Muscle &amp; Nerve"],"original-title":[],"language":"en","link":[{"URL":"https:\/\/api.wiley.com\/onlinelibrary\/tdm\/v1\/articles\/10.1002%2Fmus.25956","content-type":"application\/pdf","content-version":"vor","intended-application":"text-mining"},{"URL":"https:\/\/onlinelibrary.wiley.com\/doi\/pdf\/10.1002\/mus.25956","content-type":"application\/pdf","content-version":"vor","intended-application":"text-mining"},{"URL":"https:\/\/onlinelibrary.wiley.com\/doi\/full-xml\/10.1002\/mus.25956","content-type":"application\/xml","content-version":"vor","intended-application":"text-mining"},{"URL":"https:\/\/onlinelibrary.wiley.com\/doi\/pdf\/10.1002\/mus.25956","content-type":"unspecified","content-version":"vor","intended-application":"similarity-checking"}],"deposited":{"date-parts":[[2023,9,11]],"date-time":"2023-09-11T07:52:15Z","timestamp":1694418735000},"score":1,"resource":{"primary":{"URL":"https:\/\/onlinelibrary.wiley.com\/doi\/10.1002\/mus.25956"}},"subtitle":[],"short-title":[],"issued":{"date-parts":[[2017,9,23]]},"references-count":18,"journal-issue":{"issue":"4","published-print":{"date-parts":[[2018,4]]}},"alternative-id":["10.1002\/mus.25956"],"URL":"https:\/\/doi.org\/10.1002\/mus.25956","archive":["Portico"],"relation":{},"ISSN":["0148-639X","1097-4598"],"issn-type":[{"value":"0148-639X","type":"print"},{"value":"1097-4598","type":"electronic"}],"subject":[],"published":{"date-parts":[[2017,9,23]]}}}