{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,10,3]],"date-time":"2026-10-03T00:00:48Z","timestamp":1790985648418,"version":"4.1.0"},"reference-count":28,"publisher":"Society for Neuroscience","issue":"33","license":[{"start":{"date-parts":[[2006,2,17]],"date-time":"2006-02-17T00:00:00Z","timestamp":1140134400000},"content-version":"vor","delay-in-days":184,"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":[[2005,8,17]]},"abstract":"<jats:p>Amyotrophic lateral sclerosis (ALS), the most common motor neuron disease, is caused by a selective loss of motor neurons in the CNS. Mutations in the<jats:italic>ALS2<\/jats:italic>gene have been linked to one form of autosomal recessive juvenile onset ALS (ALS2). To investigate the pathogenic mechanisms of ALS2, we generated<jats:italic>ALS2<\/jats:italic>knock-out (<jats:italic>ALS2<\/jats:italic><jats:sup>-\/-<\/jats:sup>) mice. Although<jats:italic>ALS2<\/jats:italic><jats:sup>-\/-<\/jats:sup>mice lacked obvious developmental abnormalities, they exhibited age-dependent deficits in motor coordination and motor learning. Moreover,<jats:italic>ALS2<\/jats:italic><jats:sup>-\/-<\/jats:sup>mice showed a higher anxiety response in the open-field and elevated plus-maze tasks. Although they failed to recapitulate clinical or neuropathological phenotypes consistent with motor neuron disease by 20 months of age,<jats:italic>ALS2<\/jats:italic><jats:sup>-\/-<\/jats:sup>mice or primary cultured neurons derived from these mice were more susceptible to oxidative stress compared with wild-type controls. These observations suggest that loss of<jats:italic>ALS2<\/jats:italic>function is insufficient to cause major motor deficits or motor neuron degeneration in a mouse model but predisposes neurons to oxidative stress.<\/jats:p>","DOI":"10.1523\/jneurosci.1645-05.2005","type":"journal-article","created":{"date-parts":[[2005,8,17]],"date-time":"2005-08-17T21:43:33Z","timestamp":1124315013000},"page":"7567-7574","source":"Crossref","is-referenced-by-count":107,"title":["Loss of<i>ALS2<\/i>Function Is Insufficient to Trigger Motor Neuron Degeneration in Knock-Out Mice But Predisposes Neurons to Oxidative 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