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Abbreviations AAS: Atomic absorption spectroscopy AD: Alzheimers disease ALS: Amyotrophic lateral sclerosis Atox1, CCS, HAH1, COX17: Copper chaperones ATP7A, ATP7B, Cu-ATPases: Copper-transporting P-type ATPases A: Amyloid beta CNS: Central nervous system CopC: Bacterial peripasmic copper binding proteins COX, mitochondrial complex IV: Cytochrome c oxidase (EC 1.9.3.1) CP: Choroid plexus Ctr1, Ctr2: Copper(I) transporters CTZ: cuprizone (bis-cyclohexanoneoxalyl-dihydrazone) Cu, Zn-SOD1: Copper, zinc superoxide dismutase CuAO: Copper aminooxidase CWD: Chronic wasting disease DMT1: Divalent metal transporter D-pen: D -penicillamine EM: Electron microscopy FAAS: Flameless atomic absorption spectroscopy GABA: Gamma-aminobutyric acid GHK: SPARC fragment Gly-His-Lys GSH: Gluthation (gamma-L-glutamyl-L-cysteinylglycine) GSSG:: Gluthation disulfide HC: Huntingtons chorea Htt: Huntingtin protein ICC: Indian childhood cirrhosis ICP-AES: Inductively coupled plasma - atomic emission spectrometry IPs: Inositol phosphates Kir4.1: Inwardly rectifying K + ion channel 4.1

6B, Figs

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