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Scientists have discovered a potential treatment of copper metabolism disorders

August 1, 2018 11:07

Individuals with copper metabolism defects may soon receive more targeted treatment options due to the discovery research group led by Dr. Visala Gohila from Texas A & M AgriLife Research in College Station.

The paper in the Proceedings of the National Academy of Sciences reported that the anti-cancer drug research, eleskolmol can restore the production of complex proteins cytochrome oxidase, the critical copper-dependent enzyme required for the production of mitochondrial energy.

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Opening is vital, since copper is an essential trace metals, essential for survival. Copper is found in all body tissues, and plays a crucial role in different physiological processes, including power generation, detoxification of harmful free radicals, connective tissue maturation, biosynthesis of neurotransmitters and brain development.

Disadvantages of copper metabolism are associated with genetic disorders, such as Menksa illness that affects infants, young children and leads to deterioration of the nervous system and the state's failure to fully develop.

The study was conducted Gohil, assistant professor of biochemistry and biophysics at Texas A & M University in College Stacy, and his graduate student Shivatehoy Soma, who worked together with scientists at the Marine Biological Laboratory, University of Maryland, University of Saskatchewan in Canada, and the Department of drug detection and Life sciences in South Carolina.

"Copper - is an essential trace mineral needed for energy production of mitochondria" - Gohil said. "Inherited mutations that prevent the delivery of copper to the main mitochondrial enzyme violate energy production and lead to fatal mitochondrial disease. Currently, these disorders have no treatment. "

Gohil said that the treatment of the previous attempt of patients with these mutations by direct addition of the copper was not successful, possibly due to inefficient delivery of copper into the mitochondria.

"Thanks to a focused search of copper-containing compounds we identified eliksklamol, studied the anti-cancer drug is the most effective delivery agent of copper", - he said.

The study used multiple model organisms to test the effectiveness of salvation elesklomola copper deficiency. In particular, the model of copper deficiency in zebrafish developed by Dr. Andrew Latimer, a research scientist at the Marine Biological Laboratory in Massachusetts, was vital to show the effectiveness of eleskolmola a vertebrate. In addition, the authors have demonstrated the therapeutic potential of just elesclomol human patient cells with genetic mutations that disrupt delivery on cytochrome copper.

The study involved testing several copper-binding pharmaceutical agents for their ability to restore mitochondrial function in the yeast model. Among these compounds, they found that elieskomol unique in that it is effective at low nanomolar concentrations without causing overt toxicity at higher concentrations.

According to scientists, these findings suggest that eleskolmol can simulate the missing copper transporters, which provide the potential for treatment of human disorder of copper metabolism.

«Elesclomol undergone several clinical human trials, so our results provide an excellent opportunity to restructure the anti-cancer drug for the treatment of disorders of copper metabolism" - Gohil said.



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