{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,10,7]],"date-time":"2026-10-07T18:06:42Z","timestamp":1791396402595,"version":"4.3.3"},"reference-count":45,"publisher":"Society for Neuroscience","issue":"9","license":[{"start":{"date-parts":[[2008,8,27]],"date-time":"2008-08-27T00:00:00Z","timestamp":1219795200000},"content-version":"vor","delay-in-days":182,"URL":"https:\/\/creativecommons.org\/licenses\/by-nc-sa\/4.0\/"}],"content-domain":{"domain":[],"crossmark-restriction":false},"short-container-title":["J. Neurosci."],"published-print":{"date-parts":[[2008,2,27]]},"abstract":"<jats:p>\n                    Amyotrophic lateral sclerosis (ALS), a fatal and progressive neurodegenerative disorder characterized by weakness, muscle atrophy, and spasticity, is the most common adult-onset motor neuron disease. Although the majority of ALS cases are sporadic, \u223c5\u201310% are familial, including those linked to mutations in SOD1 (Cu\/Zn superoxide dismutase). Missense mutations in a dynactin gene (\n                    <jats:italic>DCTN1<\/jats:italic>\n                    ) encoding the p150\n                    <jats:sup>Glued<\/jats:sup>\n                    subunit of dynactin have been linked to both familial and sporadic ALS. To determine the molecular mechanism whereby mutant dynactin p150\n                    <jats:sup>Glued<\/jats:sup>\n                    causes selective degeneration of motor neurons, we generated and characterized mice expressing either wild-type or mutant human dynactin p150\n                    <jats:sup>Glued<\/jats:sup>\n                    . Neuronal expression of mutant, but not wild type, dynactin p150\n                    <jats:sup>Glued<\/jats:sup>\n                    causes motor neuron disease in these animals that are characterized by defects in vesicular transport in cell bodies of motor neurons, axonal swelling and axo-terminal degeneration. Importantly, we provide evidence that autophagic cell death is implicated in the pathogenesis of mutant p150\n                    <jats:sup>Glued<\/jats:sup>\n                    mice. This novel mouse model will be instrumental for not only clarifying disease mechanisms in ALS, but also for testing therapeutic strategies to ameliorate this devastating disease.\n                  <\/jats:p>","DOI":"10.1523\/jneurosci.4231-07.2008","type":"journal-article","created":{"date-parts":[[2008,2,27]],"date-time":"2008-02-27T17:25:02Z","timestamp":1204133102000},"page":"1997-2005","source":"Crossref","is-referenced-by-count":164,"title":["Motor Neuron Disease Occurring in a Mutant Dynactin Mouse Model Is Characterized by Defects in Vesicular Trafficking"],"prefix":"10.1523","volume":"28","author":[{"given":"Fiona M.","family":"Laird","sequence":"first","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Mohamed 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