Copper: the pathway in the body

This page shows the biochemical pathway behind the laboratory value Copper: which stations follow one another, which enzymes carry out each step and which cofactors they use. Every statement has a source. The page describes general textbook knowledge and says nothing about any individual person.

In brief

Copper is a trace element that the body absorbs from food and distributes via the liver. In enzymes it transfers electrons, for example in the respiratory chain, in superoxide dismutase and in ceruloplasmin.

The pathway step by step

1. Copper in food → Cu⁺ at the gut wall Reductases At the surface of the gut cells, reductases pass an electron to the copper. Only the reduced form Cu⁺ fits the transporter in the cell membrane. Source 1
2. Cu⁺ at the gut wall → In the gut cell CTR1 CTR1 moves Cu⁺ into the gut cell. There, glutathione and the protein metallothionein bind the copper at once, so it is not present in free form in the cell. Source 1, 5
3. In the gut cell → Copper in the blood ATP7A · ATP The pump ATP7A releases copper from the gut cell into the blood. Bound to albumin and small molecules, it travels via the portal vein to the liver. Source 1
4. Copper in the blood → Liver cell CTR1 The liver absorbs copper and distributes it onwards. What it does not use is released into the bile by the pump ATP7B; by this route copper leaves the body in the stool. Source 1
5. Liver cell → Ceruloplasmin ATP7B · ATP In the liver cell, ATP7B incorporates copper atoms into ceruloplasmin. This protein carries most of the copper in the blood and oxidises iron so that it can bind to transferrin. Source 3
6. Copper in the cell → CCS The chaperone CCS binds copper in the cytoplasm and hands it over to its target enzyme through direct contact. Source 2
7. Copper in the cell → ATOX1 ATOX1 carries copper to the two copper pumps at the Golgi apparatus. Source 2, 5
8. Copper in the cell → COX17 COX17 and other helpers bring copper into the mitochondria, the power stations of the cell. Source 4
9. CCS → SOD1 Superoxide dismutase 1 carries one copper and one zinc atom. It converts the oxygen radical superoxide into hydrogen peroxide and oxygen. Source 2
10. ATOX1 → ATP7A and ATP7B The two pumps move copper into vesicles in which new copper enzymes are assembled. When copper levels rise, they move to the cell surface and release copper to the outside. Source 5, 1
11. COX17 → Cytochrome c oxidase Complex IV contains two copper centres. At the end of the respiratory chain they transfer electrons to oxygen, forming water. Source 4
12. ATP7A and ATP7B → Copper enzymes In the vesicles, enzymes such as lysyl oxidase, which cross-links connective tissue fibres, and dopamine β-hydroxylase receive their copper atom. Source 2, 1

Cofactors in this pathway

• Glutathione — Binds copper after uptake into the cell and hands it on to the chaperones Source 1, 5In the ORY catalogue as a laboratory value: Glutathion (GSH)
• Zinc — Second metal of superoxide dismutase 1; stimulates metallothionein formation in intestinal cells Source 2, 6In the ORY catalogue as a laboratory value: Zink
• Iron — Oxidised by ceruloplasmin so that it can bind to transferrin Source 3In the ORY catalogue as a laboratory value: Eisen
• Vitamin C — Electron donor for the copper-containing dopamine β-hydroxylase Source 7In the ORY catalogue as a laboratory value: Vitamin C (Ascorbinsäure)
• ATP — Energy source for the copper pumps ATP7A and ATP7B Source 1, 5

What acts on this pathway

• Zinc — In gut cells, zinc stimulates the formation of metallothionein. This protein binds copper tightly inside the cell, so less of it passes into the blood. This relationship has been described. Source 6

Sources

1. Lutsenko S, Roy S, Tsvetkov P. Mammalian copper homeostasis: physiological roles and molecular mechanisms. Physiol Rev 2025 · PubMed 39172219
2. Robinson NJ, Winge DR. Copper metallochaperones. Annu Rev Biochem 2010 · PubMed 20205585
3. Hellman NE, Gitlin JD. Ceruloplasmin metabolism and function. Annu Rev Nutr 2002 · PubMed 12055353
4. Cobine PA, Pierrel F, Winge DR. Copper trafficking to the mitochondrion and assembly of copper metalloenzymes. Biochim Biophys Acta 2006 · PubMed 16631971
5. Lutsenko S. Dynamic and cell-specific transport networks for intracellular copper ions. J Cell Sci 2021 · PubMed 34734631
6. Avan A, Członkowska A, Gaskin S et al. The Role of Zinc in the Treatment of Wilson's Disease. Int J Mol Sci 2022 · PubMed 36012580
7. Prigge ST, Mains RE, Eipper BA et al. New insights into copper monooxygenases and peptide amidation: structure, mechanism and function. Cell Mol Life Sci 2000 · PubMed 11028916

Related pathways

• 8-OHdG — Glutathion (GSH), Zink
• Mycotoxins — Glutathion (GSH), Zink
• Borrelia — Zink, Eisen
• Calprotectin — Zink, Eisen
• Carnitine — Eisen, Vitamin C (Ascorbinsäure)

As of 2026-09-16. Draft written by Claude to schema v2; sources checked in PubMed; expert review pending
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