The cause of Lou Gehrig’s disease, severely affecting the nervous system, has so far remained a mystery (Check recent discovery about possible cause of the disease here). Finding its cure has therefore been challenging. A new study has perhaps shed light on these mysteries. The researchers claim that supplying the diseased cells with a copper compound can restore their function, thereby increasing the lifespan of those affected.
Lou Gehrig’s disease, also called amyotrophic lateral sclerosis (AML) is a disease of the nervous system which can gradually lead to paralysis of the muscles involved in voluntary movement. Its cure has not been found yet. Researchers have been carrying out many experiments in order to find a cure to it. A new study has recently shone a ray of light: it might have brought the solution to Lou Gehrig’s disease. The study has been published in the Journal of Neuroscience. The researchers interpreted their results to suggest that a copper compound might be used as a therapy for the disease.
The motor neurons responsible for relaying information from the brain to the muscles which are involved in bringing about movement slowly die such that movement becomes impossible at a point in time – paralysis occurs. People with ALS die in a very short time after having the symptoms. Generally, they die as a consequence of the respiratory complications that arise when the motor nerves have died. Only around 10 % of the patients can survive for more than a decade. For years now, scientists have been unable to explain the specific cause of the disease such that it was difficult to find a cure or therapy for ALS. Now, after having experimented on lab rats, the researchers of the new study are positive that lifespan can be extended and the degraded function of the muscles can be improved. They administrated the compound containing the copper orally into mice which had been previously genetically engineered to develop ALS. When this was done, the researchers demonstrated that the loco-motor function of those mice was enhanced.
The researchers of the new study are hopeful that this discovery could pave the way to developing a therapy for the disease. They also think that their results could be used to assist patients suffering from Parkinson’s disease as well.
ALS has been associated with the mutation of an antioxidant called SOD1, of which copper is a component. When this protein antioxidant mutates and unfolds, hence changing its specific shape, it can no more perform its function properly, which is essential to sustain life. Furthermore, its deformation entails toxicity as it becomes lethal to motor neurones. Therefore, the scientists supplied the mice with copper with the aim to stabilizing the protein so that it remains folded. The difficulty faced by the researchers was that the region of the body most in need of the copper absorbed the latter with great difficulty: it is the spinal cord which undergoes the most damage in ALS and it is also the spot where the absorption of copper is difficult. Moreover, copper is toxic to the body; its concentrations in the body are closely monitored to avoid toxic levels to build up. All of these constituted real challenges. The copper would have to be delivered into only those cells which need it in adequate amounts; not too less and not too much. The scientists managed to selectively deliver the copper compound to the affected cells.
By supplying copper to the cells of the spinal cord, the SOD1 was expected to be stabilized. The scientists did so by providing the cells of the spinal cord with the copper compound known as ‘copper (ATSM)’. When this was orally administered to the mice, their lifespans increased by 26 %. The researchers are positive that they can increase this by even more with further research.