Jim Campbell/Aero-News Network. A new treatment might allow other sufferers of motor neuron disease to survive as long as Stephen Hawking, here experiencing zero gravity

A copper compound improves the movement and lifespan of animals with the most common form of motor neuron disease, marking the first promising development in treating the high profile condition.

Amyotrophic Lateral Sclerosis (ALS), known in the US as Lou Gehrig's disease, causes the muscles to atrophy leaving sufferers wheelchair bound and eventually losing the capacity to speak, swallow and eventually breathe. Most people die within four years of diagnosis. Although ALS is one of the most common neuromuscular diseases it affects only around one person per hundred thousand each year. On the other hand, the awful nature of the symptoms and Professor Stephen Hawking's prominent struggle with the condition have kept ALS in the public eye.

Treatments exist for many of ALS's symptoms, and physical and occupational therapy can delay the progress of the condition, but the only current medication is Rilozule, which extends the life expectancy of those with the disease, and the need for ventilation support, by about 2-3 months.

So the announcement in the Journal of Neuroscience that diacetyl-bis(4-methylthiosemicarbazonato)copperII is beneficial for mice with an ALS model is significant news. A team led by Dr Blaine Roberts of Australia's Howard Florey Institute report the medication, “Increased the concentration of mutant SOD1 in ALS model mice, but paradoxically improved locomotor function and survival of the mice.”

SOD1 is an enzyme that normally has copper and zinc cofactors. Without these it unfolds and becomes lethal to motor neurone cells. The researchers found that most of the SOD1 in the spinal tissue of the ALS mice was lacking in copper. Getting copper to cells in the spinal cord is usually difficult, but the researchers found the copperII compound partially resolved this. Although the mice's SOD1 gene remained defective, teh SOD1 enzymes produced in a copper-rich environment were much less likely to cause damage to the motor neurone cells.

"The therapy we’re working toward delivers copper selectively into the cells in the spinal cord that actually need it." said author Dr Peter Crouch of the University of Melbourne. “This is a safe way to deliver a micronutrient like copper exactly where it is needed,”  

In addition to a treatment with clinical potential, the work may spur further lines of research. The paper notes, “The metal content of SOD1 may be a greater determinant of the toxicity of the protein in mutant SOD1-associated forms of ALS than the mutations themselves.”

“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 also,” said Crouch.

Despite its intimidating name diacetyl-bis(4-methylthiosemicarbazonato)copperII is cheap and easy to produce. Moreover, having already been tested as a potential cancer treatment, the process of gaining approval may be shortened.

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