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[Biochemistry](https://durgical.com/en/biochemistry/) · Topic

# Copper

Copper powers enzymes that use oxygen, build collagen and melanin and move iron; two genetic diseases show its lack and its excess.

Updated 2026-11-01

## Key facts

- Copper enzymes: cytochrome c oxidase (complex IV), lysyl oxidase (collagen and elastin cross-links), tyrosinase (melanin), dopamine β-hydroxylase, superoxide dismutase (Cu/Zn), ceruloplasmin.
- ATP7A moves copper out of the gut into blood: its failure causes Menkes disease (deficiency).
- ATP7B loads it onto ceruloplasmin and excretes it in bile: its failure causes Wilson disease (accumulation).
- Ceruloplasmin carries almost all plasma copper and oxidises iron for transferrin.

## In detail

- Sources: organ meats (liver), shellfish, nuts, seeds, cocoa, legumes.
- Absorption: in the proximal small intestine via CTR1; ATP7A pumps it to the blood side; it reaches the liver bound to albumin.
- In the hepatocyte, ATP7B incorporates it into ceruloplasmin and sends the surplus into bile, the only major route of elimination.
- Acquired deficiency: excess zinc, bariatric surgery or gastrectomy, malabsorption, copper-free parenteral nutrition. It causes anaemia (failed hephaestin and ceruloplasmin), neutropenia and a myelopathy that resembles B12 deficiency.
- Excess: Wilson disease (liver, brain, cornea), acute poisoning by copper salts (vomiting, haemolysis, liver necrosis).
- Lab: serum copper, ceruloplasmin, 24-hour urine copper; in Wilson disease, liver biopsy copper.

**Also called**
Cu, Ceruloplasmin

## Clinical notes

- Copper deficiency can be mistaken for myelodysplastic syndrome or B12 deficiency (anaemia, neutropenia, ataxia).
- Wilson disease is suspected in a young person with unexplained hepatitis or cirrhosis, tremor or psychiatric change, and Kayser-Fleischer rings.
- Menkes disease causes kinky, brittle hair, hypotonia and seizures in a baby boy.

## Related conditions

- [Wilson disease](https://durgical.com/en/conditions/wilson-disease/)
- [Menkes disease](https://durgical.com/en/conditions/menkes-disease/)

## Related

- [Iron](https://durgical.com/en/biochemistry/topics/iron/)
- [Zinc](https://durgical.com/en/biochemistry/topics/zinc/)
- [Vitamin C (ascorbic acid)](https://durgical.com/en/biochemistry/molecules/vitamin-c/)

## Test yourself

Think of the answer, then tap the question to see it.

## Which copper enzyme forms collagen cross-links?

Lysyl oxidase; without it collagen and elastin are weak (tortuous vessels and aneurysms in Menkes).

## What is the difference between ATP7A and ATP7B?

ATP7A exports copper from enterocytes to blood (failure: Menkes). ATP7B excretes it from hepatocytes into bile (failure: Wilson).

## Why does copper deficiency cause anaemia?

Hephaestin and ceruloplasmin, both copper enzymes, oxidise iron to load it onto transferrin; without them iron is trapped.

## Why is ceruloplasmin low in Wilson disease?

Without ATP7B copper is not built into ceruloplasmin; the empty protein is degraded quickly.

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Educational information, not medical advice. For any symptom, see a health professional.

Sources: [Copper in diet: MedlinePlus Medical Encyclopedia](https://medlineplus.gov/ency/article/002419.htm) (MedlinePlus, US National Library of Medicine, 2026); [ATP7A gene: MedlinePlus Genetics](https://medlineplus.gov/genetics/gene/atp7a/) (MedlinePlus, US National Library of Medicine, 2026); [ATP7B gene: MedlinePlus Genetics](https://medlineplus.gov/genetics/gene/atp7b/) (MedlinePlus, US National Library of Medicine, 2026); [Ceruloplasmin Test: MedlinePlus Medical Test](https://medlineplus.gov/lab-tests/ceruloplasmin-test/) (MedlinePlus, US National Library of Medicine, 2026); [ATP7B: Copper-transporting ATPase 2 (human)](https://www.uniprot.org/uniprotkb/P35670/entry) (UniProt, 2026); Textbook of Biochemistry for Medical Students, 6th ed. (Jaypee Brothers Medical Publishers (Vasudevan DM, Sreekumari S, Vaidyanathan K), 2011).

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