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Dysregulation of intracellular copper trafficking pathway in a mouse model of mutant copper/zinc superoxide dismutase-linked familial amyotrophic lateral sclerosis
Article first published online: 27 JUL 2009
DOI: 10.1111/j.1471-4159.2009.06310.x
© 2009 The Authors. Journal Compilation © 2009 International Society for Neurochemistry
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How to Cite
Tokuda, E., Okawa, E. and Ono, S.-i. (2009), Dysregulation of intracellular copper trafficking pathway in a mouse model of mutant copper/zinc superoxide dismutase-linked familial amyotrophic lateral sclerosis. Journal of Neurochemistry, 111: 181–191. doi: 10.1111/j.1471-4159.2009.06310.x
Publication History
- Issue published online: 14 SEP 2009
- Article first published online: 27 JUL 2009
- Received May 15, 2009; revised manuscript received July 10, 2009; accepted July 21, 2009.
References
- , , , , , and (2007) Pyrrolidine dithiocarbamate inhibits induction of immunoproteasome and decreases survival in a rat model of amyotrophic lateral sclerosis. Mol. Pharmacol. 71, 30–37.
- , , , , , and (2001) Effects of an inhibitor of poly (ADP-ribose) polymerase, desmethylselegiline, trientine, and lipoic acid in transgenic ALS mice. Exp. Neurol. 168, 419–424.
- and (2001) How to make a metalloprotein. Nat. Struct. Biol. 8, 733–734.
- , , and (1993) ALS, SOD and peroxynitrite. Nature 364, 584.
- , , , , , , , and (2006) Wild-type microglia extend survival in PU.1 knockout mice with familial amyotrophic lateral sclerosis. Proc. Natl Acad. Sci. USA 103, 16021–16026.
- , , , , , , and (2006) Onset and progression in inherited ALS determined by motor neurons and microglia. Science 312, 1389–1392.
- (2005) Anticopper therapy against cancer and diseases of inflammation and fibrosis. Drug Discov. Today 16, 1103–1109.
- , and (2004) Unraveling the mechanisms involved in motor neuron degeneration in ALS. Annu. Rev. Neurosci. 27, 723–749.
- , , et al. (2003) Wild-type nonneuronal cells extend survival of SOD1 mutant motor neurons in ALS mice. Science 302, 113–117.
- , , and (1998) Chaperone-facilitated copper binding is a property common to several classes of familial amyotrophic lateral sclerosis-linked superoxide dismutase mutants. Proc. Natl Acad. Sci. USA 95, 6361–6366.
- and (1983) Ceruloplasmin and copper in the serum of patients with amyotrophic lateral sclerosis (ALS). Neurol. Neurochir. Pol. 17, 343–346.
- , , and (1999) Aberrant copper chemistry as a major mediator of oxidative stress in a human cellular model of amyotrophic lateral sclerosis. J. Neurochem. 73, 1175–1180.
- , , , and (2003) Concentrations of Cd, Co, Cu, Fe, Mn, Rb, V, and Zn in formalin-fixed brain tissue in amyotrophic lateral sclerosis and Parkinsonism-dementia complex of Guam determined by high-resolution ICP-MS. Biol. Trace Elem. Res. 96, 39–60.
- , , et al. (1994) Motor neuron degeneration in mice that express a human Cu, Zn superoxide dismutase mutation. Science 264, 1772–1775.
- , , et al. (2008) SOD1 mutations disrupt redox-sensitive Rac regulation of NADPH oxidase in a familial ALS model. J. Clin. Invest. 118, 659–670.
- (2006) Pathogenic superoxide dismutase structure, folding, aggregation, and turnover. Curr. Opin. Chem. Biol. 10, 131–138.
- , , , and (1997) The copper chelator d-penicillamine delays onset of disease and extends survival in a transgenic mouse model of familial amyotrophic lateral sclerosis. Eur. J. Neurosci. 9, 1548–1551.
- , , , , , and (2006) Disulphide-reduced superoxide dismutase-1 in CNS of transgenic amyotrophic lateral sclerosis models. Brain 129, 451–464.
- , and (2002) Mitochondrial electron transport chain complex dysfunction in a transgenic mouse model for amyotrophic lateral sclerosis. J. Neurochem. 83, 535–545.
- , , et al. (2004) A selective requirement for copper-dependent activation of cytochrome c oxidase by Cox17p. Biochem. Biophys. Res. Commun. 324, 1379–1385.
- , , et al. (2000) The copper transport protein Atox1 promotes neuronal survival. J. Biol. Chem. 275, 580–584.
- , , , , , , and (2004) Genetically decreased spinal cord copper concentration prolongs life in a transgenic mouse model of amyotrophic lateral sclerosis. J. Neurosci. 24, 7945–7950.
- , , , , , , , and (2006) Spinal cord endoplasmic reticulum stress associated with a microsomal accumulation of mutant superoxide dismutase-1 in an ALS model. Proc. Natl Acad. Sci. USA 103, 6025–6030.
- (1996) Microglia: a sensor for pathological events in CNS. Trends Neurosci. 19, 312–318.
- , , and (2001) The copper transporter CTR1 provides an essential function in mammalian embryonic development. Proc. Natl Acad. Sci. USA 98, 6836–6841.
- , , , , and (1999) Characterization of the interaction between the Wilson and Menkes disease proteins and cytoplasmic copper chaperone, HAH1p. J. Biol. Chem. 274, 28497–28504.
- , and (2001) Essential role for mammalian copper transporter Ctr1 in copper homeostasis and embryonic development. Proc. Natl Acad. Sci. USA 98, 6842–6847.
- , , et al. (2000) Functional role of caspase-1 and caspase-3 in an ALS transgenic mouse model. Science 288, 335–339.
- , , , , , , , and (2006) Overexpression of Aβ is associated with acceleration of onset of motor impairment and superoxide dismutase 1 aggregation in an amyotrophic lateral sclerosis mouse model. Aging Cell 5, 153–165.
- (1969) Studies on the pathogenesis of hepatolenticular degeneration II. Cytochemical methods for the localization of copper. Arch. Pathol. 87, 370–379.
- (1999) Free copper ions in the cell? Science 284, 748–749.
- , , , and (1997) N-terminal domains of human copper-transporting adenosine triphosphases (the Wilson’s and Menkes disease proteins) bind copper selectivity in vivo and in vitro with stoichiometry of one copper per metal-binding repeat. J. Biol. Chem. 272, 18939–18944.
- , , , , , and (2003) The efficacy of trientine or ascorbate alone compared to that of the combined treatment with these two agents in familial amyotrophic lateral sclerosis model mice. Exp. Neurol. 179, 176–180.
- , , and (2006) The steap proteins are metalloreductases. Blood 108, 1388–1394.
- , , , , and (2001) Disease mechanisms revealed by transcription profiling in SOD1-G93A transgenic mouse spinal cord. Ann. Neurol. 50, 730–740.
- , , and (2000) Caspase-1 and -3 are sequentially activated in motor neuron death in Cu, Zn superoxide dismutase-mediated familial amyotrophic lateral sclerosis. Proc. Natl Acad. Sci. USA 97, 13901–13906.
- , , , , and (1996) Ligand-regulated transport of the Menkes copper p-type ATPase efflux pump from the Golgi apparatus to the plasma membrane: a novel mechanism of regulated trafficking. EMBO J. 15, 6084–6095.
- , , , and (1998) A C-terminal di-leucine is required for localization of the Menkes protein in the trans-Golgi network. Hum. Mol. Genet. 7, 2063–2071.
- , , , , , , , and (2002) Oxidation-induced misfolding and aggregation of superoxide dismutase and its implications for amyotrophic lateral sclerosis. J. Biol. Chem. 277, 47551–47556.
- , , et al. (1996) Motor neurons in Cu/Zn superoxide dismutase-deficient mice develop normally but exhibit enhanced cell death after axonal injury. Nat. Genet. 13, 43–47.
- , , et al. (1993) Mutations in Cu/Zn superoxide dismutase gene are associated with familial amyotrophic lateral sclerosis. Nature 362, 59–62.
- , , , , , , and (2008) Focal degeneration of astrocytes in amyotrophic lateral sclerosis. Cell Death Differ. 15, 1691–1700.
- and (2001) Amyotrophic lateral sclerosis. N. Engl. J. Med. 344, 1688–1700.
- and (2006) How do ALS-associated mutations in superoxide dismutase 1 promote aggregation of the protein? Trends Biochem. Sci. 32, 78–85.
- , , , , , and (2007) Overexpression of CCS in G93A-SOD1 mice leads to accelerated neurological deficits with severe mitochondrial pathology. Proc. Natl Acad. Sci. USA 104, 6072–6077.
- , , , , , , , and (2002) Mutant SOD1 causes motor neuron disease independent of copper chaperone-mediated copper loading. Nat. Neurosci. 5, 301–307.
- , , et al. (2002) Mammalian copper chaperone Cox17p has an essential role in activation of cytochrome c oxidase and embryonic development. Mol. Cell. Biol. 22, 7614–7621.
- , , , , and (2007) Metallothionein proteins expression, copper and zinc concentrations and lipid peroxidation level in a rodent model for amyotrophic lateral sclerosis. Toxicology 229, 33–41.
- , , , , , and (2008) Ammonium tetrathiomolybdate delays onset, prolongs survival, and slows progression of disease in a mouse model for amyotrophic lateral sclerosis. Exp. Neurol. 213, 122–128.
- , , , , and (2006) Chromogranin-mediated secretion of mutant superoxide dismutase proteins linked to amyotrophic lateral sclerosis. Nat. Neurosci. 9, 108–118.
- , , , and (2008) The endoplasmic reticulum-Golgi pathway is a target for translocation and aggregation of mutant superoxide dismutase linked to ALS. FASEB J. 22, 2476–2487.
- , , , , , , and (2003) Copper-binding-site-null SOD1 causes ALS in transgenic mice: aggregates of non-native SOD1 delineate a common feature. Hum. Mol. Genet. 12, 2753–2764.
- , and (2001) New method for quantitative mapping of metallic elements in tissue section by electron probe microanalyser with wavelength dispersive spectrometers. J. Electron Microsc. 50, 77–82.
- , , , , , , and (1996) Altered reactivity of superoxide dismutase in familial amyotrophic lateral sclerosis. Science 271, 515–518.
- , , , , , , , and (2000) Copper chaperone for superoxide dismutase is essential to activate mammalian Cu/Zn superoxide dismutase. Proc. Natl Acad. Sci. USA 97, 2886–2891.
- , , , , , , , and (2008) Astrocytes as determinants of disease progression in inherited amyotrophic lateral sclerosis. Nat. Neurosci. 11, 251–253.

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