来自俄勒冈州立大学的研究人员发现一种含铜化合物能够有效延长患有肌萎缩侧索硬化症（ALS或Lou Gehrig's disease）小鼠的生存周期。目前这一疾病尚没有有效疗法，而俄勒冈州立大学的这一成果或许将开辟治疗这一疾病的新疗法。负责这一研究的科学家Joseph Beckman介绍说含铜化合物能够帮助稳定超氧化物歧化酶（SOD）并促进其正确折叠。SOD的错误折叠正是导致ALS的主要原因之一。数据显示，利用这一疗法治疗的ALS小鼠生存期延长了26％。这一研究结果已经被发表在Journal of Neuroscience上。科学家希望通过进一步研究这一原理早日开发出能够治愈ALS的有效疗法。

详细英文报道：

Oral intake of a copper compound that's been readily available to scientists for decades has now been proven to alleviate the crippling locomotor symptoms of ALS, or Lou Gehrig's disease, in mice genetically engineered to carry the disease.

Scientists at Oregon State University (OSU) have shown that while orally dispensing the copper compound to the mice increased the concentration of mutant SOD1, responsible for ALS, it also improved the mice's locomotor function and their ability to survive.

"We believe that with further improvements, and following necessary human clinical trials for safety and efficacy, this could provide a valuable new therapy for ALS and perhaps Parkinson's disease," said Joseph Beckman, a distinguished professor of biochemistry and biophysics at the OSU College of Science, in a statement.

No treatment for ALS exists, and the research breakthrough at OSU--published on June 4 in the Journal of Neuroscience--could point toward the first therapy for the disease.

The copper, the research explains, helps to stabilize SOD and keep it "folded." The damaging and eventually fatal symptoms of ALS occur when SOD "unfolds" and becomes toxic--killing motor neurons--a result of the mutant lacking stabilizing metal cofactors.

The scientists aimed to restore enough copper to the brain and spinal cord to stabilize the SOD1 and also improve the function of the mitochondria. The brain and spinal cord are the most difficult areas of the body to do this.

The life span of the mice was improved by 26%, overall.

"The treatment increased the amount of mutant SOD, and by accepted dogma this means the animals should get worse," Blaine Roberts, lead author of the study and a research fellow at the University of Melbourne, said. "But in this case, they got a lot better. This is because we're making a targeted delivery of copper just to the cells that need it."

Funding for the research was provided by the U.S. National Institutes of Health, the Linus Pauling Institute and various organizations from Australia.