On January 28, 2016, researchers at Oregon State University (OSU) announced that they, and colleagues, had essentially stopped the progression of amyotrophic lateral sclerosis (ALS), or Lou Gehrig's disease, for nearly two years in one type of mouse model used to study the disease - allowing the mice to approach their normal lifespan. The findings, scientists indicate, are some of the most compelling ever produced in the search for a therapy for ALS, a debilitating and fatal disease, and were published online on January 27, 2015 in an open-access article in Neurobiology of Disease. The article is titled “Copper Delivery to the CNS by CuATSM Effectively Treats Motor Neuron Disease in SODG93A Mice Co-Expressing the Copper-Chaperone-for-SOD.” The new approach to ALS treatment arose from an hypothesis that sought to explain a curious observation that had previously been made in the mouse model for ALS that is based on over-expression of mutant copper/zinc superoxide dismutase (SOD). Such over-expression induces ALS-like disease in mice and this has become the most widely used model of neurodegeneration. However, no pharmaceutical agent in 20 years has extended lifespan of these ALS-model mice by more than a few weeks. The copper-chaperone-for-SOD (CCS) protein completes the maturation of SOD by inserting copper, but paradoxically human CCS causes mice co-expressing mutant SOD to die within two weeks of birth. The OSU researchers sought to explain this paradox by the hypothesis described below. The OSU researchers hyypothesized that co-expression of CCS creates a copper deficiency in the spinal cord, and therefore, the OSU team treated ALS-model mouse pups with the PET-imaging agent CuATSM, which is known to deliver copper into the CNS within minutes.
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