generation-substantially-metal-reduced-but-aldehyde-rich-aerosol-for

Generation of Substantially Metal-Reduced but Aldehyde-Rich Aerosol for E-Cigarette Toxicity Studies

When e-liquid is aerosolized in an e-cigarette for inhalation by the user, the aerosol becomes contaminated by a mixture of toxicants, including metals ...

Columbia University

Loss-of-function mutations in the copper (Cu) transporter ATP7A cause Menkes disease. Menkes is an infantile, fatal, hereditary copper-deficiency disorder that is characterized by progressive neurological injury culminating in death, typically by 3 years of age. Severe copper deficiency leads to multiple pathologies, including impaired energy generation caused by cytochrome c oxidase dysfunction in the mitochondria. Here we report that the small molecule elesclomol escorted copper to the mitochondria and increased cytochrome c oxidase levels in the brain. Through this mechanism, elesclomol prevented detrimental neurodegenerative changes and improved the survival of the mottled-brindled mouse-a murine model of severe Menkes disease. Thus, elesclomol holds promise for the treatment of Menkes and associated disorders of hereditary copper deficiency.

Andres Silva

Biography

JoVE Journal Publications

Other Publications