{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2025,10,25]],"date-time":"2025-10-25T12:00:08Z","timestamp":1761393608111},"reference-count":22,"publisher":"Wiley","issue":"1","license":[{"start":{"date-parts":[[2007,9,6]],"date-time":"2007-09-06T00:00:00Z","timestamp":1189036800000},"content-version":"vor","delay-in-days":0,"URL":"http:\/\/onlinelibrary.wiley.com\/termsAndConditions#vor"}],"content-domain":{"domain":[],"crossmark-restriction":false},"short-container-title":["Muscle and Nerve"],"published-print":{"date-parts":[[2008,1]]},"abstract":"<jats:title>Abstract<\/jats:title><jats:p>In this study we investigated a family with paramyotonia (PC) congenita caused by a Gly1306Val mutation in the voltage\u2010gated sodium\u2010channel gene <jats:italic>SCN4A<\/jats:italic>. A previous study showed that exposure to cold aggravates the muscle stiffness in patients with this mutation. However, the mechanism behind cold sensitivity and the sodium\u2010channel defect remained unclear. In order to gain a better understanding of sarcolemmal propagation in these patients, we measured muscle\u2010fiber conduction velocity (MFCV) invasively. We studied four PC patients and four healthy subjects at room temperature. After the muscle was cooled, MFCV was measured again in the two PC patients and four control subjects. MFCV was significantly lower in the PC patients at room temperature, compatible with dysfunctional sodium channels. After cooling, MFCV was significantly lower in both groups as compared with room temperature. The relative slowing was 1.4% per \u00b0C for PC patients and 1.5% per \u00b0C for healthy subjects. These results indicate that, in these PC patients, mutant and wild\u2010type sodium channels respond equally to cold exposure. Thus, MFCV is abnormal in these patients, but the aggravation of muscle stiffness cannot be explained by an abnormal sarcolemmal response to cold. Muscle Nerve, 2007<\/jats:p>","DOI":"10.1002\/mus.20885","type":"journal-article","created":{"date-parts":[[2007,9,6]],"date-time":"2007-09-06T19:17:12Z","timestamp":1189106232000},"page":"23-26","source":"Crossref","is-referenced-by-count":7,"title":["Reduced muscle\u2010fiber conduction but normal slowing after cold exposure in paramyotonia congenita"],"prefix":"10.1002","volume":"37","author":[{"given":"Paul J.","family":"Blijham","sequence":"first","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Gea","family":"Drost","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Dick F.","family":"Stegeman","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Machiel J.","family":"Zwarts","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]}],"member":"311","published-online":{"date-parts":[[2007,9,6]]},"reference":[{"key":"e_1_2_7_2_2","doi-asserted-by":"publisher","DOI":"10.1152\/japplphysiol.01009.2005"},{"key":"e_1_2_7_3_2","doi-asserted-by":"publisher","DOI":"10.1016\/0006-8993(85)90885-6"},{"key":"e_1_2_7_4_2","doi-asserted-by":"publisher","DOI":"10.1016\/S0960-8966(97)00430-6"},{"key":"e_1_2_7_5_2","doi-asserted-by":"publisher","DOI":"10.1093\/brain\/124.2.352"},{"key":"e_1_2_7_6_2","doi-asserted-by":"publisher","DOI":"10.1002\/ana.20905"},{"key":"e_1_2_7_7_2","first-page":"199","article-title":"Nerve conduction velocity: relationship of skin, subcutaneous, and intramuscular temperatures","volume":"61","author":"Halar EM","year":"1980","journal-title":"Arch Phys Med Rehabil"},{"key":"e_1_2_7_8_2","doi-asserted-by":"publisher","DOI":"10.1212\/WNL.52.7.1447"},{"key":"e_1_2_7_9_2","doi-asserted-by":"publisher","DOI":"10.1113\/jphysiol.1949.sp004388"},{"key":"e_1_2_7_10_2","doi-asserted-by":"publisher","DOI":"10.1002\/mus.880100709"},{"key":"e_1_2_7_11_2","doi-asserted-by":"publisher","DOI":"10.1113\/jphysiol.1993.sp019843"},{"key":"e_1_2_7_12_2","doi-asserted-by":"publisher","DOI":"10.1002\/ana.410390509"},{"key":"e_1_2_7_13_2","doi-asserted-by":"publisher","DOI":"10.1016\/0092-8674(92)90151-2"},{"key":"e_1_2_7_14_2","doi-asserted-by":"publisher","DOI":"10.1093\/brain\/122.6.1085"},{"key":"e_1_2_7_15_2","doi-asserted-by":"publisher","DOI":"10.1111\/j..2003.t01-1-00691.x"},{"key":"e_1_2_7_16_2","doi-asserted-by":"publisher","DOI":"10.1212\/WNL.32.8.827"},{"key":"e_1_2_7_17_2","doi-asserted-by":"publisher","DOI":"10.1152\/ajpcell.1999.277.5.C937"},{"key":"e_1_2_7_18_2","doi-asserted-by":"publisher","DOI":"10.1002\/mus.1084"},{"key":"e_1_2_7_19_2","doi-asserted-by":"publisher","DOI":"10.1111\/j.1469-7793.2001.t01-1-00579.x"},{"key":"e_1_2_7_20_2","doi-asserted-by":"publisher","DOI":"10.1212\/WNL.33.11.1453"},{"key":"e_1_2_7_21_2","doi-asserted-by":"publisher","DOI":"10.1001\/archneur.1995.00540350031007"},{"key":"e_1_2_7_22_2","doi-asserted-by":"publisher","DOI":"10.1007\/s10072-005-0461-x"},{"key":"e_1_2_7_23_2","doi-asserted-by":"publisher","DOI":"10.1002\/mus.880110213"}],"container-title":["Muscle &amp; Nerve"],"original-title":[],"language":"en","link":[{"URL":"https:\/\/api.wiley.com\/onlinelibrary\/tdm\/v1\/articles\/10.1002%2Fmus.20885","content-type":"unspecified","content-version":"vor","intended-application":"text-mining"},{"URL":"https:\/\/onlinelibrary.wiley.com\/doi\/pdf\/10.1002\/mus.20885","content-type":"unspecified","content-version":"vor","intended-application":"similarity-checking"}],"deposited":{"date-parts":[[2023,10,16]],"date-time":"2023-10-16T15:29:32Z","timestamp":1697470172000},"score":1,"resource":{"primary":{"URL":"https:\/\/onlinelibrary.wiley.com\/doi\/10.1002\/mus.20885"}},"subtitle":[],"short-title":[],"issued":{"date-parts":[[2007,9,6]]},"references-count":22,"journal-issue":{"issue":"1","published-print":{"date-parts":[[2008,1]]}},"alternative-id":["10.1002\/mus.20885"],"URL":"https:\/\/doi.org\/10.1002\/mus.20885","archive":["Portico"],"relation":{},"ISSN":["0148-639X","1097-4598"],"issn-type":[{"value":"0148-639X","type":"print"},{"value":"1097-4598","type":"electronic"}],"subject":[],"published":{"date-parts":[[2007,9,6]]}}}