You are using an unsupported browser. Please upgrade your browser to a newer version to get the best experience on Human Metabolome Database.
Record Information
Version4.0
StatusDetected and Quantified
Creation Date2006-08-16 11:03:30 UTC
Update Date2018-04-16 17:02:07 UTC
HMDB IDHMDB0000657
Secondary Accession Numbers
  • HMDB00657
Metabolite Identification
Common NameCopper
DescriptionCopper is an essential nutrient to all higher plants and animals. Physiologically, it exists as an ion in the body. In animals, it is found primarily in the bloodstream, as a cofactor in various enzymes, and in copper-based pigments. In the body, copper shifts between the cuprous (Cu1+) and cupric (Cu2+) forms, though the majority of the body's copper is in the Cu2+ form. The ability of copper to easily accept and donate electrons explains its important role in oxidation-reduction (redox) reactions and in scavenging free radicals. Copper is a critical functional component of a number of essential enzymes known as cuproenzymes. For instance, the copper-dependent enzyme, cytochrome c oxidase, plays a critical role in cellular energy production. By catalyzing the reduction of molecular oxygen (O2) to water (H2O), cytochrome c oxidase generates an electrical gradient used by the mitochondria to create the vital energy-storing molecule, ATP. Another cuproenzyme, lysyl oxidase, is required for the cross-linking of collagen and elastin, which are essential for the formation of strong and flexible connective tissue. Another cuproeznyme, Monoamine oxidase (MAO), plays a role in the metabolism of the neurotransmitters norepinephrine, epinephrine, and dopamine. MAO also functions in the degradation of the neurotransmitter serotonin, which is the basis for the use of MAO inhibitors as antidepressants. One of the most important cuproenzymes is Superoxide dismutase (SOD). SOD functions as an antioxidant by catalyzing the conversion of superoxide radicals (free radicals or ROS) to hydrogen peroxide, which can subsequently be reduced to water by other antioxidant enzymes. Two forms of SOD contain copper: 1) copper/zinc SOD is found within most cells of the body, including red blood cells, and 2) extracellular SOD is a copper-containing enzyme found at high levels in the lungs and low levels in blood plasma. In sufficient amounts, copper can be poisonous or even fatal to organisms. Copper is normally bound to cuproenzymes (such as SOD, MOA) and is thus only toxic when unsequestered and unmediated. It is believed that zinc and copper compete for absorption in the digestive tract so that a diet that is excessive in one of these minerals may result in a deficiency in the other. An imbalance of zinc and copper status might be involved in human hypertension. Furthermore, copper is found to be associated with hyperzincaemia and hypercalprotectinaemia and Wilson's disease, which are inborn errors of metabolism.
Structure
Thumb
Synonyms
ValueSource
COPPER (II) ionChEBI
Copper(II) cationChEBI
Copper, ion (cu2+)ChEBI
Cu(II)ChEBI
Cu2+ChEBI
Cu(2+)ChEBI
Cupric ionChEBI
CuHMDB
Chemical FormulaCu
Average Molecular Weight63.546
Monoisotopic Molecular Weight62.929601079
IUPAC Namecopper(2+) ion
Traditional Namecopper(2+) ion
CAS Registry Number7440-50-8
SMILES
[Cu++]
InChI Identifier
InChI=1S/Cu/q+2
InChI KeyJPVYNHNXODAKFH-UHFFFAOYSA-N
Chemical Taxonomy
DescriptionThis compound belongs to the class of inorganic compounds known as homogeneous transition metal compounds. These are inorganic compounds containing only metal atoms,with the largest atom being a transition metal atom.
KingdomInorganic compounds
Super ClassHomogeneous metal compounds
ClassHomogeneous transition metal compounds
Sub ClassNot Available
Direct ParentHomogeneous transition metal compounds
Alternative ParentsNot Available
Substituents
  • Homogeneous transition metal
Molecular FrameworkNot Available
External Descriptors
Ontology
Physiological effect

Health effect:

Disposition

Route of exposure:

Source:

Biological location:

Process

Naturally occurring process:

Role

Environmental role:

Biological role:

Physical Properties
StateSolid
Experimental Properties
PropertyValueReference
Melting Point1083 °CNot Available
Boiling PointNot AvailableNot Available
Water SolubilityNot AvailableNot Available
LogPNot AvailableNot Available
Predicted Properties
PropertyValueSource
logP0.16ChemAxon
Physiological Charge2ChemAxon
Hydrogen Acceptor Count0ChemAxon
Hydrogen Donor Count0ChemAxon
Polar Surface Area0 ŲChemAxon
Rotatable Bond Count0ChemAxon
Refractivity0 m³·mol⁻¹ChemAxon
Polarizability1.78 ųChemAxon
Number of Rings0ChemAxon
Bioavailability1ChemAxon
Rule of FiveYesChemAxon
Ghose FilterYesChemAxon
Veber's RuleYesChemAxon
MDDR-like RuleYesChemAxon
Spectra
Spectra
Spectrum TypeDescriptionSplash Key
Predicted LC-MS/MSPredicted LC-MS/MS Spectrum - 10V, Positivesplash10-03di-9000000000-59c652eccc13cc365f65View in MoNA
Predicted LC-MS/MSPredicted LC-MS/MS Spectrum - 20V, Positivesplash10-03di-9000000000-59c652eccc13cc365f65View in MoNA
Predicted LC-MS/MSPredicted LC-MS/MS Spectrum - 40V, Positivesplash10-03di-9000000000-59c652eccc13cc365f65View in MoNA
Predicted LC-MS/MSPredicted LC-MS/MS Spectrum - 10V, Negativesplash10-03di-9000000000-9acd78ab9faeb89677a7View in MoNA
Predicted LC-MS/MSPredicted LC-MS/MS Spectrum - 20V, Negativesplash10-03di-9000000000-9acd78ab9faeb89677a7View in MoNA
Predicted LC-MS/MSPredicted LC-MS/MS Spectrum - 40V, Negativesplash10-03di-9000000000-9acd78ab9faeb89677a7View in MoNA
Biological Properties
Cellular Locations
  • Cytoplasm (predicted from logP)
Biospecimen Locations
  • Blood
  • Cerebrospinal Fluid (CSF)
  • Saliva
  • Urine
Tissue Location
  • Brain
  • Erythrocyte
  • Hair
  • Intestine
  • Kidney
  • Liver
Pathways
NameSMPDB/PathwhizKEGG
AlkaptonuriaThumbThumb?image type=greyscaleThumb?image type=simpleNot Available
Aromatic L-Aminoacid Decarboxylase DeficiencyThumbThumb?image type=greyscaleThumb?image type=simpleNot Available
Beta-Alanine MetabolismThumbThumb?image type=greyscaleThumb?image type=simpleMap00410
Carnosinuria, carnosinemiaThumbThumb?image type=greyscaleThumb?image type=simpleNot Available
Catecholamine BiosynthesisThumbThumb?image type=greyscaleThumb?image type=simpleMap00350
Normal Concentrations
BiospecimenStatusValueAgeSexConditionReferenceDetails
BloodDetected and Quantified14.71645(14.20635-15.22336) uMAdult (>18 years old)BothNormal
    • Report on Human B...
details
BloodDetected and Quantified14.5 +/- 3.1 uMElderly (>65 years old)BothNormal details
BloodDetected and Quantified0.89 (0.39) uMAdult (>18 years old)MaleNormal details
BloodDetected and Quantified13.376-24.392 uMAdult (>18 years old)Not SpecifiedNormal details
BloodDetected and Quantified1.416-7.239 uMNewborn (0-30 days old)BothNormal details
BloodDetected and Quantified11.0156-24.392 uMNewborn (0-30 days old)BothNormal details
BloodDetected and Quantified12.589-22.0313 uMChildren (1 - 13 years old)FemaleNormal details
BloodDetected and Quantified4.56 +/- 1.83 uMNewborn (0-30 days old)BothNormal
    • Geigy Scientific ...
details
BloodDetected and Quantified20.3 (13.1 - 27.4) uMChildren (1-13 years old)BothNormal
    • Geigy Scientific ...
details
BloodDetected and Quantified17.0 (10.2 - 29.0) uMAdult (>18 years old)FemaleNormal
    • Geigy Scientific ...
details
BloodDetected and Quantified23.7 (11.3 - 27.0) uMAdult (>18 years old)FemaleNormal
    • Geigy Scientific ...
details
BloodDetected and Quantified17.2 (12.7 - 21.6) uMAdult (>18 years old)MaleNormal
    • Geigy Scientific ...
details
BloodDetected and Quantified37.6 (23.6 - 49.9) uMAdult (>18 years old)FemaleNormal
    • Geigy Scientific ...
details
BloodDetected and Quantified11.8 (7.5 - 16.1) uMAdult (>18 years old)FemaleNormal
    • Geigy Scientific ...
details
BloodDetected and Quantified12-22 uMAdult (>18 years old)BothNormal details
BloodDetected and Quantified18.9 uMAdult (>18 years old)FemaleNormal
    • Geigy Scientific ...
details
BloodDetected and Quantified17.2 uMAdult (>18 years old)MaleNormal
    • Geigy Scientific ...
details
BloodDetected and Quantified12.7-22.0 uMChildren (1-13 years old)Not SpecifiedNormal details
BloodDetected and Quantified10.0-22.0 uMAdult (>18 years old)Female
Normal
details
BloodDetected and Quantified10.0-22.0 uMAdult (>18 years old)Male
Normal
details
BloodDetected and Quantified10.229-25.179 uMInfant (0-1 year old)Not SpecifiedNormal details
BloodDetected and Quantified15.06127(14.09512-15.95434) uMNot AvailableBothNormal
    • Report on Human B...
details
Cerebrospinal Fluid (CSF)Detected and Quantified1.7 +/- 1.4 uMAdult (>18 years old)Not SpecifiedNormal details
Cerebrospinal Fluid (CSF)Detected and Quantified1.69 +/- 1.54 uMElderly (>65 years old)BothNormal details
Cerebrospinal Fluid (CSF)Detected and Quantified2.253 +/- 3.118 uMAdult (>18 years old)Not SpecifiedNormal details
Cerebrospinal Fluid (CSF)Detected and Quantified0.26 (0.24-0.28) uMChildren (1-13 years old)BothNormal
    • Geigy Scientific ...
details
SalivaDetected and Quantified2.74 (1.66) uMAdult (>18 years old)MaleNormal details
SalivaDetected and Quantified0.234 +/- 0.127 uMAdult (>18 years old)BothNormal
    • Zerihun T. Dame, ...
details
SalivaDetected and Quantified4.1-61.7 uMAdult (>18 years old)Both
Normal
details
SalivaDetected and Quantified4.1-57.6 uMAdult (>18 years old)Both
Normal
details
SalivaDetected and Quantified4.1-57.6 uMAdult (>18 years old)Both
Normal
details
SalivaDetected and Quantified0.315-0.708 uMChildren (1-13 years old)BothNormal
    • Gian Paolo Sighin...
details
SalivaDetected and Quantified2.833 +/- 4.406 uMAdult (>18 years old)Male
Normal
details
SalivaDetected and Quantified2.990 +/- 4.878 uMAdult (>18 years old)Male
Normal
details
SalivaDetected and Quantified3.619 +/- 1.574 uMAdult (>18 years old)Not SpecifiedNormal
    • Natheer H. Al-Raw...
details
SalivaDetected and Quantified0.0241 +/- 0.0209 uMAdult (>18 years old)Male
Normal
details
SalivaDetected and Quantified0.0323 +/- 0.0203 uMAdult (>18 years old)Male
Normal
details
SalivaDetected and Quantified0.0219 +/- 0.00708 uMAdult (>18 years old)Male
Normal
details
SalivaDetected and Quantified0.0271 +/- 0.0183 uMAdult (>18 years old)Male
Normal
details
UrineDetected and Quantified0.01994(0.01940-0.05192) umol/mmol creatinineNot AvailableBothNormal
    • Report on Human B...
details
UrineDetected and Quantified0.02174 (0.01815-0.02551) umol/mmol creatinineAdult (>18 years old)BothNormal
    • Report on Human B...
details
UrineDetected and Quantified0.00467-0.0533 umol/mmol creatinineChildren (1-13 years old)Not SpecifiedNormal details
UrineDetected and Quantified0.0163 (0.0006-0.1099) umol/mmol creatinineAdult (>18 years old)Both
Normal
details
UrineDetected and Quantified0.025 (0.013-0.044) umol/mmol creatinineAdult (>18 years old)MaleNormal
    • Geigy Scientific ...
    • West Cadwell, N.J...
    • Basel, Switzerlan...
details
UrineDetected and Quantified0.019 (0.0092-0.038) umol/mmol creatinineAdult (>18 years old)FemaleNormal
    • Geigy Scientific ...
    • West Cadwell, N.J...
    • Basel, Switzerlan...
details
UrineDetected and Quantified0.0380 (0.0107-0.119) umol/mmol creatinineAdult (>18 years old)BothNormal
    • Geigy Scientific ...
details
UrineDetected and Quantified0.0313 umol/mmol creatinineChildren (1 - 13 years old)Not SpecifiedNormal
    • Geigy Scientific ...
details
Abnormal Concentrations
BiospecimenStatusValueAgeSexConditionReferenceDetails
BloodDetected and Quantified0.283-12.668 uMChildren (1-13 years old)Both
Wilson's disease
    • Wilson's disease ...
details
BloodDetected and Quantified22.96 +/- 7.64 uMAdult (>18 years old)BothMultiple sclerosis details
BloodDetected and Quantified15.70 +/- 3.80 uMAdult (>18 years old)BothParkinson's disease details
BloodDetected and Quantified1.34 (0.57) uMAdult (>18 years old)MaleOral submucous fibrosis (OSMF) details
BloodDetected and Quantified6.295-7.868 uMAdult (>18 years old)Female
Aceruloplasminemia
details
BloodDetected and Quantified25.0842 uMChildren (1 - 13 years old)FemaleHyperzincaemia and Hypercalprotectinaemia details
BloodDetected and Quantified1.574-8.340 uMInfant (0-1 year old)Both
Menkes syndrome
details
BloodDetected and Quantified4.564-10.701 uMAdult (>18 years old)Both
Wilson's disease
details
BloodDetected and Quantified6.6 uMAdult (>18 years old)MaleOccipital Horn Syndrome details
BloodDetected and Quantified11.4 uMChildren (1-13 years old)MaleOccipital Horn Syndrome details
BloodDetected and Quantified2.4-2.8 uMChildren (1-13 years old)MaleMental retardation, enteropathy, deafness, peripheral neuropathy, ichthyosis, and keratoderma details
BloodDetected and Quantified15.4 +/- 3.9 uMElderly (>65 years old)BothAlzheimer's disease details
BloodDetected and Quantified4.878-11.645 uMInfant (0-1 year old)MaleCongenital cataracts, hearing loss, and neurodegeneration details
Cerebrospinal Fluid (CSF)Detected and Quantified0.944-1.464 uMAdult (>18 years old)Both
Wilson's disease
details
Cerebrospinal Fluid (CSF)Detected and Quantified1.39 +/- 1.02 uMElderly (>65 years old)BothAlzheimer's disease details
SalivaDetected and Quantified4.65 (1.59) uMAdult (>18 years old)MaleOral submucous fibrosis (OSMF) details
SalivaDetected and Quantified2.0458 +/- 1.416 uMAdult (>18 years old)Not SpecifiedOral squamous cell carcinoma
    • Natheer H. Al-Raw...
details
SalivaDetected and Quantified2.518 +/- 0.944 uMAdult (>18 years old)Not Specified
Oral squamous cell carcinoma
    • Natheer H. Al-Raw...
details
SalivaDetected and Quantified1.526 +/- 0.944 uMAdult (>18 years old)Not Specified
Oral squamous cell carcinoma
    • Natheer H. Al-Raw...
details
UrineDetected and Quantified0.0163-1.0491 umol/mmol creatinineChildren (1-13 years old)Both
Wilson's disease
    • Wilson's disease ...
details
UrineDetected and Quantified0.17 umol/mmol creatinineChildren (1-13 years old)MaleMental retardation, enteropathy, deafness, peripheral neuropathy, ichthyosis, and keratoderma details
UrineDetected and Quantified>0.0420 umol/mmol creatinineChildren (1-13 years old)BothWilson's disease details
UrineDetected and Quantified>0.105 umol/mmol creatinineAdult (>18 years old)BothWilson's disease details
UrineDetected and Quantified0.162-0.938 umol/mmol creatinineAdult (>18 years old)Both
Wilson's disease
details
Associated Disorders and Diseases
Disease References
Alzheimer's disease
  1. Molina JA, Jimenez-Jimenez FJ, Aguilar MV, Meseguer I, Mateos-Vega CJ, Gonzalez-Munoz MJ, de Bustos F, Porta J, Orti-Pareja M, Zurdo M, Barrios E, Martinez-Para MC: Cerebrospinal fluid levels of transition metals in patients with Alzheimer's disease. J Neural Transm (Vienna). 1998;105(4-5):479-88. [PubMed:9720975 ]
  2. Bocca B, Forte G, Petrucci F, Pino A, Marchione F, Bomboi G, Senofonte O, Giubilei F, Alimonti A: Monitoring of chemical elements and oxidative damage in patients affected by Alzheimer's disease. Ann Ist Super Sanita. 2005;41(2):197-203. [PubMed:16244393 ]
Multiple sclerosis
  1. Forte G, Visconti A, Santucci S, Ghazaryan A, Figa-Talamanca L, Cannoni S, Bocca B, Pino A, Violante N, Alimonti A, Salvetti M, Ristori G: Quantification of chemical elements in blood of patients affected by multiple sclerosis. Ann Ist Super Sanita. 2005;41(2):213-6. [PubMed:16244395 ]
Parkinson's disease
  1. Forte G, Alimonti A, Pino A, Stanzione P, Brescianini S, Brusa L, Sancesario G, Violante N, Bocca B: Metals and oxidative stress in patients with Parkinson's disease. Ann Ist Super Sanita. 2005;41(2):189-95. [PubMed:16244392 ]
Wilson's disease
  1. Patil M, Sheth KA, Krishnamurthy AC, Devarbhavi H: A review and current perspective on Wilson disease. J Clin Exp Hepatol. 2013 Dec;3(4):321-36. doi: 10.1016/j.jceh.2013.06.002. Epub 2013 Jul 6. [PubMed:25755520 ]
  2. Weisner B, Hartard C, Dieu C: CSF copper concentration: a new parameter for diagnosis and monitoring therapy of Wilson's disease with cerebral manifestation. J Neurol Sci. 1987 Jun;79(1-2):229-37. [PubMed:3612170 ]
  3. Sócio D.A., et al. (2010). Wilson's disease in children and adolescents: diagnosis and treatment. Rev Paul Pediatr 28(2):134-40.. Rev Paul Pediatr.
Hyperzincaemia and hypercalprotectinaemia
  1. Isidor B, Poignant S, Corradini N, Fouassier M, Quartier P, Roth J, Picherot G: Hyperzincemia and hypercalprotectinemia: unsuccessful treatment with tacrolimus. Acta Paediatr. 2009 Feb;98(2):410-2. doi: 10.1111/j.1651-2227.2008.01092.x. Epub 2008 Nov 4. [PubMed:18983438 ]
Aceruloplasminemia
  1. Roberti Mdo R, Borges Filho HM, Goncalves CH, Lima FL: Aceruloplasminemia: a rare disease - diagnosis and treatment of two cases. Rev Bras Hematol Hemoter. 2011;33(5):389-92. doi: 10.5581/1516-8484.20110104. [PubMed:23049345 ]
Congenital cataracts, hearing loss, and neurodegeneration
  1. Horvath R, Freisinger P, Rubio R, Merl T, Bax R, Mayr JA, Shawan, Muller-Hocker J, Pongratz D, Moller LB, Horn N, Jaksch M: Congenital cataract, muscular hypotonia, developmental delay and sensorineural hearing loss associated with a defect in copper metabolism. J Inherit Metab Dis. 2005;28(4):479-92. doi: 10.1007/s10545-005-0479-x. [PubMed:15902551 ]
Mental retardation, enteropathy, deafness, peripheral neuropathy, ichthyosis, and keratoderma
  1. Martinelli D, Travaglini L, Drouin CA, Ceballos-Picot I, Rizza T, Bertini E, Carrozzo R, Petrini S, de Lonlay P, El Hachem M, Hubert L, Montpetit A, Torre G, Dionisi-Vici C: MEDNIK syndrome: a novel defect of copper metabolism treatable by zinc acetate therapy. Brain. 2013 Mar;136(Pt 3):872-81. doi: 10.1093/brain/awt012. Epub 2013 Feb 18. [PubMed:23423674 ]
Occipital Horn Syndrome
  1. Kuivaniemi H, Peltonen L, Palotie A, Kaitila I, Kivirikko KI: Abnormal copper metabolism and deficient lysyl oxidase activity in a heritable connective tissue disorder. J Clin Invest. 1982 Mar;69(3):730-3. [PubMed:6120954 ]
Associated OMIM IDs
  • 126200 (Multiple sclerosis)
  • 168600 (Parkinson's disease)
  • 277900 (Wilson's disease)
  • 194470 (Hyperzincaemia and hypercalprotectinaemia)
  • 604290 (Aceruloplasminemia)
  • 614482 (Congenital cataracts, hearing loss, and neurodegeneration)
  • 609313 (Mental retardation, enteropathy, deafness, peripheral neuropathy, ichthyosis, and keratoderma)
  • 304150 (Occipital Horn Syndrome)
  • 104300 (Alzheimer's disease)
DrugBank IDDB09130
Phenol Explorer Compound IDNot Available
FoodDB IDFDB003582
KNApSAcK IDNot Available
Chemspider ID25221
KEGG Compound IDC00070
BioCyc IDCUCL2
BiGG IDNot Available
Wikipedia LinkCopper
METLIN IDNot Available
PubChem Compound27099
PDB IDCU
ChEBI ID29036
References
Synthesis ReferenceNot Available
Material Safety Data Sheet (MSDS)Download (PDF)
General References
  1. Kedzierska E: [Concentrations of selected bioelements and toxic metals and their influence on health status of children and youth residing in Szczecin]. Ann Acad Med Stetin. 2003;49:131-43. [PubMed:15552844 ]
  2. Koury JC, de Olilveria AV Jr, Portella ES, de Olilveria CF, Lopes GC, Donangelo CM: Zinc and copper biochemical indices of antioxidant status in elite athletes of different modalities. Int J Sport Nutr Exerc Metab. 2004 Jun;14(3):358-72. [PubMed:15256695 ]
  3. Hoogenraad TU: Paradigm shift in treatment of Wilson's disease: zinc therapy now treatment of choice. Brain Dev. 2006 Apr;28(3):141-6. Epub 2006 Feb 7. [PubMed:16466879 ]
  4. Dib N, Valsesia E, Malinge MC, Mauras Y, Misrahi M, Cales P: Late onset of Wilson's disease in a family with genetic haemochromatosis. Eur J Gastroenterol Hepatol. 2006 Jan;18(1):43-7. [PubMed:16357618 ]
  5. Kodama H, Sato E, Gu YH, Shiga K, Fujisawa C, Kozuma T: Effect of copper and diethyldithiocarbamate combination therapy on the macular mouse, an animal model of Menkes disease. J Inherit Metab Dis. 2005;28(6):971-8. [PubMed:16435190 ]
  6. Cengiz B, Soylemez F, Ozturk E, Cavdar AO: Serum zinc, selenium, copper, and lead levels in women with second-trimester induced abortion resulting from neural tube defects: a preliminary study. Biol Trace Elem Res. 2004 Mar;97(3):225-35. [PubMed:14997023 ]
  7. Langner C, Denk H: Wilson disease. Virchows Arch. 2004 Aug;445(2):111-8. Epub 2004 Jun 17. [PubMed:15205951 ]
  8. Kitzberger R, Madl C, Ferenci P: Wilson disease. Metab Brain Dis. 2005 Dec;20(4):295-302. [PubMed:16382340 ]
  9. Chen D, Cui QC, Yang H, Dou QP: Disulfiram, a clinically used anti-alcoholism drug and copper-binding agent, induces apoptotic cell death in breast cancer cultures and xenografts via inhibition of the proteasome activity. Cancer Res. 2006 Nov 1;66(21):10425-33. [PubMed:17079463 ]
  10. Briviba K, Schnabele K, Rechkemmer G, Bub A: Supplementation of a diet low in carotenoids with tomato or carrot juice does not affect lipid peroxidation in plasma and feces of healthy men. J Nutr. 2004 May;134(5):1081-3. [PubMed:15113949 ]
  11. Pizent A, Jurasovic J, Telisman S: Serum calcium, zinc, and copper in relation to biomarkers of lead and cadmium in men. J Trace Elem Med Biol. 2003;17(3):199-205. [PubMed:14968933 ]
  12. Squitti R, Barbati G, Rossi L, Ventriglia M, Dal Forno G, Cesaretti S, Moffa F, Caridi I, Cassetta E, Pasqualetti P, Calabrese L, Lupoi D, Rossini PM: Excess of nonceruloplasmin serum copper in AD correlates with MMSE, CSF [beta]-amyloid, and h-tau. Neurology. 2006 Jul 11;67(1):76-82. [PubMed:16832081 ]
  13. Odland JO, Nieboer E, Romanova N, Thomassen Y: Elements in placenta and pregnancy outcome in arctic and subarctic areas. Int J Circumpolar Health. 2004 May;63(2):169-87. [PubMed:15253483 ]
  14. Venelinov TI, Davies IM, Beattie JH: Dialysis-Chelex method for determination of exchangeable copper in human plasma. Anal Bioanal Chem. 2004 Jul;379(5-6):777-80. Epub 2004 Feb 26. [PubMed:14991216 ]
  15. Attri S, Sharma N, Jahagirdar S, Thapa BR, Prasad R: Erythrocyte metabolism and antioxidant status of patients with Wilson disease with hemolytic anemia. Pediatr Res. 2006 Apr;59(4 Pt 1):593-7. [PubMed:16549536 ]
  16. Jablonska-Kaszewska I, Dabrowska E, Drobinska Jurowiecka A, Falkiewicz B: Treatment of Wilson's disease. Med Sci Monit. 2003 Aug;9 Suppl 3:5-8. [PubMed:15156602 ]
  17. Daniel KG, Harbach RH, Guida WC, Dou QP: Copper storage diseases: Menkes, Wilsons, and cancer. Front Biosci. 2004 Sep 1;9:2652-62. [PubMed:15358588 ]
  18. Aoki T: [Genetic disorders of copper transport--diagnosis and new treatment for the patients of Wilson's disease]. No To Hattatsu. 2005 Mar;37(2):99-109. [PubMed:15773321 ]
  19. Meng Y, Miyoshi I, Hirabayashi M, Su M, Mototani Y, Okamura T, Terada K, Ueda M, Enomoto K, Sugiyama T, Kasai N: Restoration of copper metabolism and rescue of hepatic abnormalities in LEC rats, an animal model of Wilson disease, by expression of human ATP7B gene. Biochim Biophys Acta. 2004 Nov 5;1690(3):208-19. [PubMed:15511628 ]
  20. Gorter RW, Butorac M, Cobian EP: Examination of the cutaneous absorption of copper after the use of copper-containing ointments. Am J Ther. 2004 Nov-Dec;11(6):453-8. [PubMed:15543084 ]

Only showing the first 10 proteins. There are 51 proteins in total.

Enzymes

General function:
Involved in oxidoreductase activity
Specific function:
This is a copper-containing oxidase that functions in the formation of pigments such as melanins and other polyphenolic compounds. Catalyzes the rate-limiting conversions of tyrosine to DOPA, DOPA to DOPA-quinone and possibly 5,6-dihydroxyindole to indole-5,6 quinone.
Gene Name:
TYR
Uniprot ID:
P14679
Molecular weight:
60392.69
General function:
Involved in monooxygenase activity
Specific function:
Bifunctional enzyme that catalyzes 2 sequential steps in C-terminal alpha-amidation of peptides. The monooxygenase part produces an unstable peptidyl(2-hydroxyglycine) intermediate that is dismutated to glyoxylate and the corresponding desglycine peptide amide by the lyase part. C-terminal amidation of peptides such as neuropeptides is essential for full biological activity.
Gene Name:
PAM
Uniprot ID:
P19021
Molecular weight:
108402.425
General function:
Involved in monooxygenase activity
Specific function:
Conversion of dopamine to noradrenaline.
Gene Name:
DBH
Uniprot ID:
P09172
Molecular weight:
69064.45
General function:
Involved in copper ion binding
Specific function:
Catalyzes the degradation of compounds such as putrescine, histamine, spermine, and spermidine, substances involved in allergic and immune responses, cell proliferation, tissue differentiation, tumor formation, and possibly apoptosis. Placental DAO is thought to play a role in the regulation of the female reproductive function.
Gene Name:
ABP1
Uniprot ID:
P19801
Molecular weight:
85377.1
General function:
Involved in copper ion binding
Specific function:
Cell adhesion protein that participates in lymphocyte recirculation by mediating the binding of lymphocytes to peripheral lymph node vascular endothelial cells in an L-selectin-independent fashion. Has a monoamine oxidase activity. May play a role in adipogenesis.
Gene Name:
AOC3
Uniprot ID:
Q16853
Molecular weight:
84621.27
General function:
Involved in copper ion binding
Specific function:
Has a monoamine oxidase activity with substrate specificity for 2-phenylethylamine and tryptamine. May play a role in adipogenesis. May be a critical modulator of signal transmission in retina.
Gene Name:
AOC2
Uniprot ID:
O75106
Molecular weight:
80515.11
General function:
Involved in oxidoreductase activity
Specific function:
Stores iron in a soluble, non-toxic, readily available form. Important for iron homeostasis. Has ferroxidase activity. Iron is taken up in the ferrous form and deposited as ferric hydroxides after oxidation.
Gene Name:
FTMT
Uniprot ID:
Q8N4E7
Molecular weight:
27537.885
General function:
Involved in oxidoreductase activity
Specific function:
Ceruloplasmin is a blue, copper-binding (6-7 atoms per molecule) glycoprotein. It has ferroxidase activity oxidizing Fe(2+) to Fe(3+) without releasing radical oxygen species. It is involved in iron transport across the cell membrane. Provides Cu(2+) ions for the ascorbate-mediated deaminase degradation of the heparan sulfate chains of GPC1. May also play a role in fetal lung development or pulmonary antioxidant defense (By similarity).
Gene Name:
CP
Uniprot ID:
P00450
Molecular weight:
122204.45
General function:
Involved in copper ion binding
Specific function:
Responsible for the post-translational oxidative deamination of peptidyl lysine residues in precursors to fibrous collagen and elastin. In addition to cross-linking of extracellular matrix proteins, may have a direct role in tumor suppression.
Gene Name:
LOX
Uniprot ID:
P28300
Molecular weight:
46943.67
General function:
Involved in iron ion binding
Specific function:
Cytochrome c oxidase is the component of the respiratory chain that catalyzes the reduction of oxygen to water. Subunits 1-3 form the functional core of the enzyme complex. CO I is the catalytic subunit of the enzyme. Electrons originating in cytochrome c are transferred via the copper A center of subunit 2 and heme A of subunit 1 to the bimetallic center formed by heme A3 and copper B.
Gene Name:
MT-CO1
Uniprot ID:
P00395
Molecular weight:
57040.91

Transporters

General function:
Involved in ATP binding
Specific function:
Involved in the export of copper out of the cells, such as the efflux of hepatic copper into the bile.
Gene Name:
ATP7B
Uniprot ID:
P35670
Molecular weight:
157261.34
General function:
Involved in ATP binding
Specific function:
May supply copper to copper-requiring proteins within the secretory pathway, when localized in the trans-Golgi network. Under conditions of elevated extracellular copper, it relocalized to the plasma membrane where it functions in the efflux of copper from cells.
Gene Name:
ATP7A
Uniprot ID:
Q04656
Molecular weight:
163372.275
General function:
Involved in copper ion transmembrane transporter activity
Specific function:
Involved in high-affinity copper uptake
Gene Name:
SLC31A1
Uniprot ID:
O15431
Molecular weight:
21090.5
General function:
Involved in copper ion transmembrane transporter activity
Specific function:
Involved in low-affinity copper uptake (Potential)
Gene Name:
SLC31A2
Uniprot ID:
O15432
Molecular weight:
15681.3

Only showing the first 10 proteins. There are 51 proteins in total.