Copper delivery to the CNS by CuATSM effectively treats motor neuron disease in SODG93A mice co-expressing the Copper-Chaperone-for-SOD
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- 作者：Jared R. Williamsa ; Emiliano Triasb ; c ; Pamela R. Beilbya ; Nathan I. Lopeza ; Edwin M. Labuta ; C. Samuel Bradforda ; Blaine R. Robertsd ; Erin J. McAllumd ; e ; Peter J. Crouchd ; e ; Timothy W. Rhoadsa ; 1 ; Cliff Pereiraf ; Marjatta Song ; Jeffrey L. Elliottg ; Maria Clara Francoh ; Alvaro G. Esté ; vezh ; Luis Barbeitoc ; Joseph S. Beckmana ; joe.beckman@oregonstate.edu" class="auth_mail" title="E-mail the corresponding author
- 关键词：SOD1 ; Lou Gehrig ; Amyotrophic lateral sclerosis ; Superoxide dismutase ; CCS
- 刊名：Neurobiology of Disease
- 出版年：2016
- 出版时间：May 2016
- 年：2016
- 卷：89
- 期：Complete
- 页码：1-9
- 全文大小：1472 K
文摘
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The copper ligand CuATSM extends life in high-expressing SODG93A mice by up to 25%.
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CuATSM in high-expressing SODG93A mice co-expressing CCS survive for 20 months (500%).
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Motor neuron disease can be restarted and then stopped by controlling CuATSM treatment.
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CCS plus copper nearly doubles the amount of SOD protein in mouse spinal cord.
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The vast majority of the mutant SOD is found in its mature and active form, Cu, Zn SOD.