{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,10,4]],"date-time":"2026-10-04T06:45:14Z","timestamp":1791096314065,"version":"4.1.0"},"reference-count":44,"publisher":"Wiley","issue":"3","license":[{"start":{"date-parts":[[2011,12,12]],"date-time":"2011-12-12T00:00:00Z","timestamp":1323648000000},"content-version":"vor","delay-in-days":0,"URL":"http:\/\/onlinelibrary.wiley.com\/termsAndConditions#vor"}],"funder":[{"DOI":"10.13039\/501100000024","name":"Canadian Institutes of Health Research","doi-asserted-by":"publisher","award":["86725"],"award-info":[{"award-number":["86725"]}],"id":[{"id":"10.13039\/501100000024","id-type":"DOI","asserted-by":"publisher"}]},{"DOI":"10.13039\/100005202","name":"Muscular Dystrophy Association","doi-asserted-by":"publisher","award":["MDA93897"],"award-info":[{"award-number":["MDA93897"]}],"id":[{"id":"10.13039\/100005202","id-type":"DOI","asserted-by":"publisher"}]}],"content-domain":{"domain":["faseb.onlinelibrary.wiley.com"],"crossmark-restriction":true},"short-container-title":["The FASEB Journal"],"published-print":{"date-parts":[[2012,3]]},"abstract":"<jats:p>\n                    Intermediate filaments serve important structural roles, but other cellular functions are increasingly recognized. This study demonstrated normal function of the low\u2010molecular\u2010weight neurofilament protein (NFL) in mitochondrial dynamics and disruption in Charcot\u2010Marie\u2010Tooth disease (CMT) due to mutations in the\n                    <jats:italic>Nefl<\/jats:italic>\n                    gene. In motor neurons of spinal cord cultured from\n                    <jats:italic>Nefl<\/jats:italic>\n                    \u2010knockout mice, mitochondrial length and the rate of fusion were decreased concomitant with increased motility. These parameters were normalized after expression of NFL\n                    <jats:sup>wt<\/jats:sup>\n                    on the\n                    <jats:italic>\n                      Nefl\n                      <jats:sup>\u2010\/\u2010<\/jats:sup>\n                    <\/jats:italic>\n                    background, but not by overexpression of the profusion protein, mitofusin 2 (MFN2). The effects of CMT\u2010causing NFL mutants bore similarities to and differences from\n                    <jats:italic>Nefl<\/jats:italic>\n                    knockout. In the early phase of toxicity before disruption of the neurofilament network, NFL\n                    <jats:sup>Q333P<\/jats:sup>\n                    and NFL\n                    <jats:sup>P8R<\/jats:sup>\n                    integrated into neurofilaments and had effects on mitochondria similar to those with\n                    <jats:italic>Nefl<\/jats:italic>\n                    knockout. The reduction of fusion rate by NFL\n                    <jats:sup>Q333P<\/jats:sup>\n                    was partly due to interference with the function of the profusion protein MFN2, which is mutated in CMT2A, functionally linking these forms of CMT. In the later phase of toxicity, mitochondria essentially stopped moving in neurons expressing NFL mutants, probably a consequence of cytoskeletal disruption. Overall, the data point to important functions of neurofilaments in mitochondrial dynamics as well as primary involvement in CMT2E\/1F.\u2014Gentil, B. J. Minotti, S., Beange, M., Baloh, R. H., Julien, J.\u2010P., Durham, H. D. Normal role of the low\u2010molecular\u2010weight neurofilament protein in mitochondrial dynamics and disruption in Charcot\u2010Marie\u2010Tooth disease.\n                    <jats:italic>FASEB J.<\/jats:italic>\n                    26, 1194\u20101203 (2012).\n                    <jats:ext-link xmlns:xlink=\"http:\/\/www.w3.org\/1999\/xlink\" xlink:href=\"http:\/\/www.fasebj.org\">www.fasebj.org<\/jats:ext-link>\n                  <\/jats:p>","DOI":"10.1096\/fj.11-196345","type":"journal-article","created":{"date-parts":[[2011,12,13]],"date-time":"2011-12-13T00:56:04Z","timestamp":1323737764000},"page":"1194-1203","update-policy":"https:\/\/doi.org\/10.1002\/crossmark_policy","source":"Crossref","is-referenced-by-count":62,"title":["Normal role of the low\u2010molecular\u2010weight neurofilament protein in mitochondrial dynamics and disruption in Charcot\u2010Marie\u2010Tooth disease"],"prefix":"10.1096","volume":"26","author":[{"given":"Benoit 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Neurology Washington University School of Medicine St. Louis Missouri USA"},{"name":"Hope Center for Neurological Diseases Washington University School of Medicine St. Louis Missouri USA"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Jean\u2010Pierre","family":"Julien","sequence":"additional","affiliation":[{"name":"Centre de Recherche du Centre Hospitalier Universitaire de Qu\u00e9bec Universit\u00e9 Laval Qu\u00e9bec Quebec Canada"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Heather D.","family":"Durham","sequence":"additional","affiliation":[{"name":"Department of Neurology and Neurosurgery McGill University Montreal Quebec Canada"},{"name":"Montreal Neurological Institute McGill University Montreal Quebec 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