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Record Information
Version3.6
Creation Date2006-08-16 11:03:30 UTC
Update Date2013-02-08 18:19:25 UTC
HMDB IDHMDB00657
Secondary Accession NumbersNone
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.
Structure
Thumb
Synonyms
  1. Copper
  2. Cu
Chemical FormulaCu
Average Molecular Weight63.546
Monoisotopic Molecular Weight62.929601079
IUPAC Namecopper(2+) ion
Traditional IUPAC Namecopper(2+)
CAS Registry Number7440-50-8
SMILES
[Cu++]
InChI Identifier
InChI=1S/Cu/q+2
InChI KeyJPVYNHNXODAKFH-UHFFFAOYSA-N
Chemical Taxonomy
KingdomInorganic Compounds
Super ClassHomogeneous Metal Compounds
ClassHomogeneous Transition Metal Compounds
Sub ClassN/A
Other Descriptors
  • a cation(Cyc)
  • copper cation(ChEBI)
  • divalent metal cation(ChEBI)
  • monoatomic dication(ChEBI)
Substituents
  • N/A
Direct ParentHomogeneous Transition Metal Compounds
Ontology
StatusDetected and Quantified
Origin
  • Food
  • Plant
  • Toxin/Pollutant
Biofunction
  • Enzyme co-factor
  • Essential minerals
ApplicationNot Available
Cellular locations
  • Cytoplasm (predicted from logP)
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 area0ChemAxon
rotatable bond count0ChemAxon
refractivity0ChemAxon
polarizability1.78ChemAxon
Spectra
SpectraNot Available
Biological Properties
Cellular Locations
  • Cytoplasm (predicted from logP)
Biofluid Locations
  • Blood
  • Cerebrospinal Fluid (CSF)
  • Urine
Tissue Location
  • Brain
  • Erythrocyte
  • Hair
  • Intestine
  • Kidney
  • Liver
PathwaysNot Available
Normal Concentrations
BiofluidStatusValueAgeSexConditionReferenceDetails
BloodDetected and Quantified14.71645(14.20635-15.22336) uMAdult (>18 years old)BothNormal
    • Report on Human B...
details
BloodDetected and Quantified22.01 (14.16-29.89) uMAdult (>18 years old)BothNormal details
BloodDetected and Quantified14.5 +/- 3.1 uMElderly (>65 years old)BothNormal 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 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
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 Quantified0.0163 (0.0006-0.1099) umol/mmol creatinineAdult (>18 years old)Both
Normal
details
UrineDetected and Quantified0.02 (0.00-0.04) umol/mmol creatinineAdult (>18 years old)BothNormal 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
Abnormal Concentrations
BiofluidStatusValueAgeSexConditionReferenceDetails
BloodDetected and Quantified7.0 (1.5-12.7) uMChildren (1-13 years old)BothMenkes syndrome 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 Quantified7.87 (3.15-12.58) uMChildren (1-13 years old)BothWilson's disease details
BloodDetected and Quantified15.4 +/- 3.9 uMElderly (>65 years old)BothAlzheimer's disease details
Cerebrospinal Fluid (CSF)Detected and Quantified1.39 +/- 1.02 uMElderly (>65 years old)BothAlzheimer's disease details
UrineDetected and Quantified0.57 (0.10-1.14) umol/mmol creatinineChildren (1-13 years old)BothWilson'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. 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
Menkes disease
  1. MetaGene
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. MetaGene
Associated OMIM IDs
DrugBank IDNot Available
DrugBank Metabolite IDNot Available
Phenol Explorer Compound IDNot Available
Phenol Explorer Metabolite IDNot Available
FoodDB IDFDB003582
KNApSAcK IDNot Available
Chemspider ID25221
KEGG Compound IDC00070
BioCyc IDCUCL2
BiGG IDNot Available
Wikipedia LinkCopper
NuGOwiki LinkHMDB00657
Metagene LinkHMDB00657
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 50 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
General function:
Involved in nucleotide binding
Specific function:
May be involved in cell growth. Probably acts as a terminal oxidase of plasma electron transport from cytosolic NAD(P)H via hydroquinones to acceptors at the cell surface. Hydroquinone oxidase activity alternates with a protein disulfide- thiol interchange/oxidoreductase activity which may control physical membrane displacements associated with vesicle budding or cell enlargement. The activities oscillate with a period length of 22 minutes and play a role in control of the ultradian cellular biological clock
Gene Name:
ENOX2
Uniprot ID:
Q16206
Molecular weight:
70081.5
General function:
Involved in superoxide dismutase activity
Specific function:
Destroys superoxide anion radicals which are normally produced within the cells and which are toxic to biological systems.
Gene Name:
SOD2
Uniprot ID:
P04179
Molecular weight:
24750.015
General function:
Involved in binding
Specific function:
N-APP binds TNFRSF21 triggering caspase activation and degeneration of both neuronal cell bodies (via caspase-3) and axons (via caspase-6)
Gene Name:
APP
Uniprot ID:
P05067
Molecular weight:
86942.7
General function:
Involved in metal ion binding
Specific function:
Destroys radicals which are normally produced within the cells and which are toxic to biological systems.
Gene Name:
SOD1
Uniprot ID:
P00441
Molecular weight:
15935.685
General function:
Involved in calcium ion binding
Specific function:
Appears to regulate cell growth through interactions with the extracellular matrix and cytokines. Binds calcium and copper, several types of collagen, albumin, thrombospondin, PDGF and cell membranes. There are two calcium binding sites; an acidic domain that binds 5 to 8 Ca(2+) with a low affinity and an EF-hand loop that binds a Ca(2+) ion with a high affinity
Gene Name:
SPARC
Uniprot ID:
P09486
Molecular weight:
34631.9
General function:
Involved in cell adhesion
Specific function:
Central regulator of hemostasis. It serves as a critical cofactor for the prothrombinase activity of factor Xa that results in the activation of prothrombin to thrombin
Gene Name:
F5
Uniprot ID:
P12259
Molecular weight:
251701.2
General function:
Involved in copper ion binding
Specific function:
Oxidation of 5,6-dihydroxyindole-2-carboxylic acid (DHICA) into indole-5,6-quinone-2-carboxylic acid. May regulate or influence the type of melanin synthesized
Gene Name:
TYRP1
Uniprot ID:
P17643
Molecular weight:
60723.8
General function:
Involved in transport
Specific function:
Binds copper, nickel, and fatty acids as well as, and bilirubin less well than, serum albumin. Only a small percentage (less than 2%) of the human AFP shows estrogen-binding properties
Gene Name:
AFP
Uniprot ID:
P02771
Molecular weight:
68676.9
General function:
Involved in metal ion binding
Specific function:
Binds and deliver cytosolic copper to the copper ATPase proteins. May be important in cellular antioxidant defense.
Gene Name:
ATOX1
Uniprot ID:
O00244
Molecular weight:
7401.575
General function:
Involved in serine-type endopeptidase activity
Specific function:
Catalyzes the first step in the proteolytic activation of the sterol regulatory element-binding proteins (SREBPs). Other known substrates are BDNF and ATF6. Cleaves after hydrophobic or small residues, provided that Arg or Lys is in position P4. Cleaves known substrates after Arg-Ser-Val-Leu (SERBP-2), Arg-His- Leu-Leu (ATF6), Arg-Gly-Leu-Thr (BDNF) and its own propeptide after Arg-Arg-Leu-Leu
Gene Name:
MBTPS1
Uniprot ID:
Q14703
Molecular weight:
117747.7
General function:
Involved in Mo-molybdopterin cofactor biosynthetic process
Specific function:
Microtubule-associated protein involved in membrane protein-cytoskeleton interactions. It is thought to anchor the inhibitory glycine receptor (GLYR) to subsynaptic microtubules (By similarity). Catalyzes two steps in the biosynthesis of the molybdenum cofactor. In the first step, molybdopterin is adenylated. Subsequently, molybdate is inserted into adenylated molybdopterin and AMP is released.
Gene Name:
GPHN
Uniprot ID:
Q9NQX3
Molecular weight:
79747.635
General function:
Involved in metal ion binding
Specific function:
Protect the extracellular space from toxic effect of reactive oxygen intermediates by converting superoxide radicals into hydrogen peroxide and oxygen.
Gene Name:
SOD3
Uniprot ID:
P08294
Molecular weight:
25850.675
General function:
Involved in monooxygenase activity
Specific function:
Not Available
Gene Name:
MOXD1
Uniprot ID:
Q6UVY6
Molecular weight:
69651.9
General function:
Involved in copper 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. Subunit 2 transfers the electrons from cytochrome c via its binuclear copper A center to the bimetallic center of the catalytic subunit 1
Gene Name:
MT-CO2
Uniprot ID:
P00403
Molecular weight:
25564.7
General function:
Involved in serine-type endopeptidase activity
Specific function:
Likely to represent a ubiquitous endoprotease activity within constitutive secretory pathways and capable of cleavage at the RXXX[KR]R consensus motif
Gene Name:
PCSK7
Uniprot ID:
Q16549
Molecular weight:
86246.4
General function:
Involved in binding
Specific function:
The gamma-CTF peptide, C30, is a potent enhancer of neuronal apoptosis
Gene Name:
APLP1
Uniprot ID:
P51693
Molecular weight:
72175.8
General function:
Involved in metal ion binding
Specific function:
Delivers copper to copper zinc superoxide dismutase (SOD1)
Gene Name:
CCS
Uniprot ID:
O14618
Molecular weight:
29040.4
General function:
Involved in identical protein binding
Specific function:
Inhibits TNF-induced NFKB1 activation. May function to facilitate biliary copper excretion within hepatocytes
Gene Name:
COMMD1
Uniprot ID:
Q8N668
Molecular weight:
21177.9
General function:
Involved in copper ion binding
Specific function:
Exerts its effect at some terminal stage of cytochrome c oxidase synthesis, probably by being involved in the insertion of the copper B into subunit I
Gene Name:
COX11
Uniprot ID:
Q9Y6N1
Molecular weight:
31429.7
General function:
Involved in copper ion binding
Specific function:
Copper chaperone for cytochrome c oxidase (COX). Binds two copper ions and deliver them to the Cu(A) site of COX
Gene Name:
COX17
Uniprot ID:
Q14061
Molecular weight:
6915.0
General function:
Involved in metal ion binding
Specific function:
Required for mitochondrial cytochrome c oxidase (COX) assembly and respiration. Binds copper. May be involved in copper trafficking and distribution to COX and SOD1
Gene Name:
CMC1
Uniprot ID:
Q7Z7K0
Molecular weight:
12489.5
General function:
Involved in copper ion binding
Specific function:
Involved in copper homeostasis
Gene Name:
CUTC
Uniprot ID:
Q9NTM9
Molecular weight:
29340.7
General function:
Involved in nucleotide binding
Specific function:
Probably acts as a terminal oxidase of plasma electron transport from cytosolic NAD(P)H via hydroquinones to acceptors at the cell surface. Hydroquinone oxidase activity alternates with a protein disulfide-thiol interchange/oxidoreductase activity which may control physical membrane displacements associated with vesicle budding or cell enlargement. The activities oscillate with a period length of 24 minutes and play a role in control of the ultradian cellular biological clock
Gene Name:
ENOX1
Uniprot ID:
Q8TC92
Molecular weight:
73347.8
General function:
Involved in copper ion binding
Specific function:
May function as a ferroxidase for ferrous (II) to ferric ion (III) conversion and may be involved in copper transport and homeostasis. Implicated in iron homeostasis and may mediate iron efflux associated to ferroportin 1
Gene Name:
HEPH
Uniprot ID:
Q9BQS7
Molecular weight:
130447.8
General function:
Involved in copper ion binding
Specific function:
May function as a ferroxidase and may be involved in copper transport and homeostasis
Gene Name:
HEPHL1
Uniprot ID:
Q6MZM0
Molecular weight:
131601.7
General function:
Involved in copper ion binding
Specific function:
Active on elastin and collagen substrates
Gene Name:
LOXL1
Uniprot ID:
Q08397
Molecular weight:
63109.3
General function:
Involved in copper ion binding
Specific function:
Mediates the post-translational oxidative deamination of lysine residues on target proteins leading to the formation of deaminated lysine (allysine). When secreted in extracellular matrix, promotes cross-linking of extracellular matrix proteins by mediating oxidative deamination of peptidyl lysine residues in precursors to fibrous collagen and elastin. Acts as a regulator of sprouting angiogenesis, probably via collagen IV scaffolding. When nuclear, acts as a transcription corepressor and specifically mediates deamination of trimethylated 'Lys-4' of histone H3 (H3K4me3), a specific tag for epigenetic transcriptional activation. Involved in epithelial to mesenchymal transition (EMT) via interaction with SNAI1 and participates in repression of E-cadherin, probably by mediating deamination of histone H3. Also involved in E-cadherin repression following hypoxia, a hallmark of epithelial to mesenchymal transition believed to amplify tumor aggressiveness, suggesting that it may play a role in tumor progression. Acts as a regulator of chondrocyte differentiation, probably by regulating expression of factors that control chondrocyte differentiation.
Gene Name:
LOXL2
Uniprot ID:
Q9Y4K0
Molecular weight:
86724.305
General function:
Involved in copper ion binding
Specific function:
Not Available
Gene Name:
LOXL3
Uniprot ID:
P58215
Molecular weight:
83165.9
General function:
Involved in copper ion binding
Specific function:
May modulate the formation of a collagenous extracellular matrix
Gene Name:
LOXL4
Uniprot ID:
Q96JB6
Molecular weight:
84483.1
General function:
Involved in monooxygenase activity
Specific function:
Not Available
Gene Name:
MOXD2
Uniprot ID:
A6NHM9
Molecular weight:
56319.0
General function:
Involved in metal ion binding
Specific function:
Binds heavy metals. Contains three zinc and three copper atoms per polypeptide chain and only a negligible amount of cadmium. Inhibits survival and neurite formation of cortical neurons in vitro
Gene Name:
MT3
Uniprot ID:
P25713
Molecular weight:
6926.9
General function:
Involved in metal ion binding
Specific function:
Seems to bind zinc and copper. Could play a special role in regulating zinc metabolism during the differentiation of stratified epithelia
Gene Name:
MT4
Uniprot ID:
P47944
Molecular weight:
6508.7
General function:
Involved in copper ion binding
Specific function:
Thought to play a role in cellular copper homeostasis, mitochondrial redox signaling or insertion of copper into the active site of COX
Gene Name:
SCO1
Uniprot ID:
O75880
Molecular weight:
33813.7
General function:
Involved in copper ion binding
Specific function:
Acts as a copper chaperone, transporting copper to the Cu(A) site on the cytochrome c oxidase subunit II (COX2)
Gene Name:
SCO2
Uniprot ID:
O43819
Molecular weight:
29809.7
General function:
Involved in iron ion binding
Specific function:
Endosomal ferrireductase required for efficient transferrin-dependent iron uptake in erythroid cells. Participates in erythroid iron homeostasis by reducing Fe(3+) to Fe(2+). Can also reduce of Cu(2+) to Cu(1+), suggesting that it participates in copper homeostasis. Uses NAD(+) as acceptor. May play a role downstream of p53/TP53 to interface apoptosis and cell cycle progression. Indirectly involved in exosome secretion by facilitating the secretion of proteins such as TCTP
Gene Name:
STEAP3
Uniprot ID:
Q658P3
Molecular weight:
54600.1
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. Also plays a role in delivery of iron to cells. Mediates iron uptake in capsule cells of the developing kidney (By similarity).
Gene Name:
FTH1
Uniprot ID:
P02794
Molecular weight:
21225.47
General function:
Involved in oxidoreductase activity
Specific function:
Not Available
Gene Name:
CP
Uniprot ID:
A5PL27
Molecular weight:
122204.4

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 50 proteins. There are 51 proteins in total.