Loss of ALS2 function is insufficient to trigger motor neuron degeneration in knock-out mice but predisposes neurons to oxidative stress

Huaibin Cai, Xian Lin, Chengsong Xie, Fiona M. Laird, Chen Lai, Hongjin Wen, Hsueh Cheng Chiang, Hoon Shim, Mohamed H. Farah, Ahmet Hoke, Donald L. Price, Philip C. Wong

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102 引文 斯高帕斯(Scopus)

摘要

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 ALS2 gene have been linked to one form of autosomal recessive juvenile onset ALS (ALS2). To investigate the pathogenic mechanisms of ALS2, we generated ALS2 knock-out (ALS2-/-) mice. Although ALS2-/- mice lacked obvious developmental abnormalities, they exhibited age-dependent deficits in motor coordination and motor learning. Moreover, ALS2-/- 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, ALS2-/- 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 ALS2 function is insufficient to cause major motor deficits or motor neuron degeneration in a mouse model but predisposes neurons to oxidative stress.

原文English
頁(從 - 到)7567-7574
頁數8
期刊Journal of Neuroscience
25
發行號33
DOIs
出版狀態Published - 2005 8月 17

All Science Journal Classification (ASJC) codes

  • 一般神經科學

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