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33746889803
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Copper homeostasis in eukaryotes: Teetering on a tightrope
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Balamurugana K, Schaffner W. Copper homeostasis in eukaryotes: teetering on a tightrope. Biochim Biophys Acta 2006; 1763:737-746.
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(2006)
Biochim Biophys Acta
, vol.1763
, pp. 737-746
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Balamurugana, K.1
Schaffner, W.2
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3
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33747849534
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Nose Y, Kim BE, Thiele DJ. Ctr1 drives intestinal copper absorption and is essential for growth, iron metabolism, and neonatal cardiac function. Cell Metab 2006; 4:235-244. This is an elegant and important analysis of the mechanisms of copper absorption utilizing genetic technology in mice. The tissue specific loss of Ctr1 reveals a striking phenotype of copper deficiency in neonates and demonstrates the critical role for this transporter in intestinal copper uptake.
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Nose Y, Kim BE, Thiele DJ. Ctr1 drives intestinal copper absorption and is essential for growth, iron metabolism, and neonatal cardiac function. Cell Metab 2006; 4:235-244. This is an elegant and important analysis of the mechanisms of copper absorption utilizing genetic technology in mice. The tissue specific loss of Ctr1 reveals a striking phenotype of copper deficiency in neonates and demonstrates the critical role for this transporter in intestinal copper uptake.
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4
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31544454133
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Kuo YM, Gybina AA, Pyatskowit JW, et al. Copper transport protein (Ctr1) levels in mice are tissue specific and dependent on copper status. J Nutr 2006; 136:21-26. This is a detailed analysis of Ctr1 expression in the mouse and the first evidence that the copper status of the animal affects expression of Ctr1. These findings suggest a level of regulation of Ctr1 directly related to a role in copper acquisition.
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Kuo YM, Gybina AA, Pyatskowit JW, et al. Copper transport protein (Ctr1) levels in mice are tissue specific and dependent on copper status. J Nutr 2006; 136:21-26. This is a detailed analysis of Ctr1 expression in the mouse and the first evidence that the copper status of the animal affects expression of Ctr1. These findings suggest a level of regulation of Ctr1 directly related to a role in copper acquisition.
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6
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33750326197
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Nose Y, Rees EM, Thiele DJ. Structure of the Ctr1 copper trans'PORE'ter reveals novel architecture. Trends Biochem Sci 2006; 31:604-607. This review of the human Ctr1 structure is placed in the physiological context of copper transport and reconciled with the considerable biochemical data on the function of this protein.
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Nose Y, Rees EM, Thiele DJ. Structure of the Ctr1 copper trans'PORE'ter reveals novel architecture. Trends Biochem Sci 2006; 31:604-607. This review of the human Ctr1 structure is placed in the physiological context of copper transport and reconciled with the considerable biochemical data on the function of this protein.
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7
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33745713097
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Anemia and neutropenia associated with copper deficiency of unclear etiology
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Harless W, Crowell E, Abraham J. Anemia and neutropenia associated with copper deficiency of unclear etiology. Am J Hematol 2006; 81:546-549.
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(2006)
Am J Hematol
, vol.81
, pp. 546-549
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Harless, W.1
Crowell, E.2
Abraham, J.3
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9
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33749234649
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Motor neuron disease associated with copper deficiency
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Weihl CC, Lopate G. Motor neuron disease associated with copper deficiency. Muscle Nerve 2006; 34:789-793.
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(2006)
Muscle Nerve
, vol.34
, pp. 789-793
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Weihl, C.C.1
Lopate, G.2
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10
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33747395126
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Relapsing hypocupraemic myelopathy requiring high-dose oral copper replacement
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This report of a case of relapsing hypocupraemic myelopathy responsive to increased doses of copper gives caution that standard doses of copper may not be sufficient for all patients
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Prodan CI, Bottomley SS, Holland NR, et al. Relapsing hypocupraemic myelopathy requiring high-dose oral copper replacement. J Neurol Neurosurg Psychiatry 2006; 77:1092-1093. This report of a case of relapsing hypocupraemic myelopathy responsive to increased doses of copper gives caution that standard doses of copper may not be sufficient for all patients.
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(2006)
J Neurol Neurosurg Psychiatry
, vol.77
, pp. 1092-1093
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Prodan, C.I.1
Bottomley, S.S.2
Holland, N.R.3
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11
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11244346808
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Zinc-induced copper deficiency: A report of three cases initially recognized on bone marrow examination
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Willis MS, Monaghan SA, Miller ML, et al. Zinc-induced copper deficiency: a report of three cases initially recognized on bone marrow examination. Am J Clin Pathol 2005; 123:125-131.
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(2005)
Am J Clin Pathol
, vol.123
, pp. 125-131
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Willis, M.S.1
Monaghan, S.A.2
Miller, M.L.3
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12
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33646699146
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Neuropathy progressing to myeloneuropathy 20 years after partial gastrectomy
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Everett CM, Matharu M, Gawler J. Neuropathy progressing to myeloneuropathy 20 years after partial gastrectomy. Neurology 2006; 66:1451.
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(2006)
Neurology
, vol.66
, pp. 1451
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Everett, C.M.1
Matharu, M.2
Gawler, J.3
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13
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33749254859
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Tan JC, Burns, Jones HR. Severe ataxia, myelopathy, and peripheral neuropathy due to acquired copper deficiency in a patient with history of gastrectomy. JPEN J Parenter Enteral Nutr 2006; 30:446-450.
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Tan JC, Burns, Jones HR. Severe ataxia, myelopathy, and peripheral neuropathy due to acquired copper deficiency in a patient with history of gastrectomy. JPEN J Parenter Enteral Nutr 2006; 30:446-450.
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14
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32344452060
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Kumar N, Ahlskog JE, Klein CJ, et al. Imaging features of copper deficiency myelopathy: a study of 25 cases. Neuroradiology 2006; 48:78-83. This is a review of the magnetic resonance imaging findings in 25 patients with acquired copper deficiency. This study provided neuroradiologic confirmation of the clinical symptoms in many patients and indicated that the diagnosis should be considered when a long segment of symmetric dorsal spinal cord T2-hyperintensity is identified.
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Kumar N, Ahlskog JE, Klein CJ, et al. Imaging features of copper deficiency myelopathy: a study of 25 cases. Neuroradiology 2006; 48:78-83. This is a review of the magnetic resonance imaging findings in 25 patients with acquired copper deficiency. This study provided neuroradiologic confirmation of the clinical symptoms in many patients and indicated that the diagnosis should be considered when a long segment of symmetric dorsal spinal cord T2-hyperintensity is identified.
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15
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33846696924
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Prodan CI, Bottomley SS, Vincent AS, et al. Hypocupremia associated with prior vitamin B(12) deficiency. Am J Hematol 2006; Sep 19 [Epub ahead of print]. The neurologic features of copper deficiency are similar to those observed with vitamin B12 deficiency. For this reason, the authors conducted a pilot study that suggested that a significant number of elderly patients with prior diagnosis of vitamin B12 deficiency also have undiagnosed hypocupremia. This suggests a previously unappreciated role for copper in this pathology and perhaps an interaction between these cofactors.
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Prodan CI, Bottomley SS, Vincent AS, et al. Hypocupremia associated with prior vitamin B(12) deficiency. Am J Hematol 2006; Sep 19 [Epub ahead of print]. The neurologic features of copper deficiency are similar to those observed with vitamin B12 deficiency. For this reason, the authors conducted a pilot study that suggested that a significant number of elderly patients with prior diagnosis of vitamin B12 deficiency also have undiagnosed hypocupremia. This suggests a previously unappreciated role for copper in this pathology and perhaps an interaction between these cofactors.
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16
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33746558633
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Atp7a determines a hierarchy of copper metabolism essential for notochord development
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Utilizing a unique zebrafish model of copper deficiency the authors combined chemical and forward genetics to demonstrate a hierarchy of copper distribution in the embryo that is of direct relevance to the biology of human birth defects
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Mendelsohn BA, Yin C, Johnson SL, et al. Atp7a determines a hierarchy of copper metabolism essential for notochord development. Cell Metab 2006; 4:155-162. Utilizing a unique zebrafish model of copper deficiency the authors combined chemical and forward genetics to demonstrate a hierarchy of copper distribution in the embryo that is of direct relevance to the biology of human birth defects.
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(2006)
Cell Metab
, vol.4
, pp. 155-162
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Mendelsohn, B.A.1
Yin, C.2
Johnson, S.L.3
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18
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30044445876
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Essential roles in development and pigmentation for the Drosophila copper transporter DmATP7
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Norgate M, Lee E, Southon A, et al. Essential roles in development and pigmentation for the Drosophila copper transporter DmATP7. Mol Biol Cell 2006; 17:475-484.
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(2006)
Mol Biol Cell
, vol.17
, pp. 475-484
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Norgate, M.1
Lee, E.2
Southon, A.3
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19
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33746727678
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Niciu MJ, Ma XM, ElMeskini R, et al. Developmental changes in the expression of ATP7A during a critical period in postnatal neurodevelopment. Neuroscience 2006; 139:947-964. An atp7a-specific antibody was used to study the neurodevelopmental expression and localization of this protein in the developing mouse brain. Atp7a increases in expression in CA2 hippocampal pyramidal and cerebellar Purkinje neurons during development and this precisely-regulated neurodevelopmental expression correlates precisely with the therapeutic window for effective copper treatment in patients with Menkes disease.
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Niciu MJ, Ma XM, ElMeskini R, et al. Developmental changes in the expression of ATP7A during a critical period in postnatal neurodevelopment. Neuroscience 2006; 139:947-964. An atp7a-specific antibody was used to study the neurodevelopmental expression and localization of this protein in the developing mouse brain. Atp7a increases in expression in CA2 hippocampal pyramidal and cerebellar Purkinje neurons during development and this precisely-regulated neurodevelopmental expression correlates precisely with the therapeutic window for effective copper treatment in patients with Menkes disease.
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20
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24144458246
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El Meskini R, Cline LB, Eipper BA, et al. The developmentally regulated expression of Menkes protein ATP7A suggests a role in axon extension and synaptogenesis. Dev Neurosci 2005; 27:333-348. The expression of atp7a was investigated using the murine olfactory system as a neurodevelopmental model. During development, atp7a expression shifted to extending axons, peaking prior to synaptogenesis. Similarly, after injury-stimulated neurogenesis, atp7a expression was increased in neurons and axons preceding synaptogenesis. These results support a role for atp7a in axon extension, a finding of relevance to the severe neurodegeneration characteristic of patients with Menkes disease.
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El Meskini R, Cline LB, Eipper BA, et al. The developmentally regulated expression of Menkes protein ATP7A suggests a role in axon extension and synaptogenesis. Dev Neurosci 2005; 27:333-348. The expression of atp7a was investigated using the murine olfactory system as a neurodevelopmental model. During development, atp7a expression shifted to extending axons, peaking prior to synaptogenesis. Similarly, after injury-stimulated neurogenesis, atp7a expression was increased in neurons and axons preceding synaptogenesis. These results support a role for atp7a in axon extension, a finding of relevance to the severe neurodegeneration characteristic of patients with Menkes disease.
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21
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23044500932
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Downregulation of myelination, energy, and translational genes in Menkes disease brain
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Liu PC, Chen YW, Centeno JA, et al. Downregulation of myelination, energy, and translational genes in Menkes disease brain. Mol Genet Metab 2005; 85:291-300.
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(2005)
Mol Genet Metab
, vol.85
, pp. 291-300
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Liu, P.C.1
Chen, Y.W.2
Centeno, J.A.3
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22
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12144257164
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Schlief ML, Craig AM, Gitlin JD. NMDA receptor activation mediates copper homeostasis in hippocampal neurons. J Neurosci 2005; 25:239-246. These studies demonstrate a critical role for atp7a in the availability of an NMDA receptor-dependent, releasable pool of copper in hippocampal neurons, suggesting a unique mechanism linking copper homeostasis and neuronal activation within the CNS.
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Schlief ML, Craig AM, Gitlin JD. NMDA receptor activation mediates copper homeostasis in hippocampal neurons. J Neurosci 2005; 25:239-246. These studies demonstrate a critical role for atp7a in the availability of an NMDA receptor-dependent, releasable pool of copper in hippocampal neurons, suggesting a unique mechanism linking copper homeostasis and neuronal activation within the CNS.
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33749534285
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Schlief M, West T, Craig A, et al. Role of the Menkes copper transporting atpase in NMDA receptor-mediated neuronal toxicity. Proc Natl Acad Sci U S A 2006; 103:14919-14924. These studies indicated that copper chelation exacerbates NMDA-mediated excitotoxic cell death in primary hippocampal neurons, whereas the addition of copper is specifically protective and that this protective effect of copper depends on endogenous nitric oxide production in hippocampal neurons. Atp7a is required for these copper-dependent effects, indicating a unique connection between copper homeostasis and NMDA receptor activity that is of broad relevance to the processes of synaptic plasticity and excitotoxic cell death in patients with Menkes disease.
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Schlief M, West T, Craig A, et al. Role of the Menkes copper transporting atpase in NMDA receptor-mediated neuronal toxicity. Proc Natl Acad Sci U S A 2006; 103:14919-14924. These studies indicated that copper chelation exacerbates NMDA-mediated excitotoxic cell death in primary hippocampal neurons, whereas the addition of copper is specifically protective and that this protective effect of copper depends on endogenous nitric oxide production in hippocampal neurons. Atp7a is required for these copper-dependent effects, indicating a unique connection between copper homeostasis and NMDA receptor activity that is of broad relevance to the processes of synaptic plasticity and excitotoxic cell death in patients with Menkes disease.
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24
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33646856612
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Copper homeostasis in the CNS: A novel link between the NMDA receptor and copper homeostasis in the hippocampus
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Schlief ML, Gitlin JD. Copper homeostasis in the CNS: a novel link between the NMDA receptor and copper homeostasis in the hippocampus. Mol Neurobiol 2006; 33:81-90.
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(2006)
Mol Neurobiol
, vol.33
, pp. 81-90
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Schlief, M.L.1
Gitlin, J.D.2
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