Article
Deregulation of subcellular biometal homeostasis through loss of the metal transporter, Zip7, in a childhood neurodegenerative disorder.
Acta neuropathologica communications - 28 Feb 2014
Grubman Alexandra, Lidgerwood Grace E, Duncan Clare, Bica Laura, Tan Jiang-Li, Parker Sarah J, Caragounis Aphrodite, Meyerowitz Jodi, Volitakis Irene, Moujalled Diane, Liddell Jeffrey R, Hickey James L, Horne Malcolm, Longmuir Shoshanah, Koistinaho Jari, Donnelly Paul S, Crouch Peter J, Tammen Imke, White Anthony R, Kanninen Katja M
Abstract excerpt
BACKGROUND: Aberrant biometal metabolism is a key feature of neurodegenerative disorders including Alzheimer's and Parkinson's diseases. Metal modulating compounds are promising therapeutics for neurodegeneration, but their mechanism of action remains poorly understood. Neuronal ceroid lipofuscinoses (NCLs), caused by mutations in CLN genes, are fatal childhood neurodegenerative lysosomal storage diseases without...
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