Article
Prenatal treatment of mosaic mice (Atp7a mo-ms) mouse model for Menkes disease, with copper combined by dimethyldithiocarbamate (DMDTC).
PloS one - 1 Jan 2012
Lenartowicz Małgorzata, Krzeptowski Wojciech, Koteja Paweł, Chrząścik Katarzyna, Møller Lisbeth Birk
Abstract excerpt
Menkes disease is a fatal neurodegenerative disorder in infants caused by mutations in the gene ATP7A which encodes a copper (Cu) transporter. Defects in ATP7A lead to accumulated copper in the small intestine and kidneys and to copper deficiencies in the brain and the liver. The copper level in the kidney in postnatal copper-treated Menkes patients may reach toxic levels. The mouse model, mosaic Atp7a (mo-ms)...
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