Ceruloplasmin
| Ceruloplasmin | ||
|---|---|---|
|
||
| Belt / surface model with copper (blue) according to PDB 1KCW | ||
| Properties of human protein | ||
| Mass / length primary structure | 132 kilodaltons / 1046 amino acids | |
| Cofactor | 8 Cu 2+ | |
| Identifier | ||
| Gene names | CP ; CP-2 | |
| External IDs | ||
| Enzyme classification | ||
| EC, category | 1.16.3.1 , oxidoreductase | |
| Response type | oxidation | |
| Substrate | 4 Fe 2+ + 4 H + + O 2 | |
| Products | 4 Fe 3+ + 2 H 2 O | |
| Occurrence | ||
| Homology family | Ceruloplasmin | |
| Parent taxon | Newcomers | |
| Orthologue | ||
| human | House mouse | |
| Entrez | 1356 | 12870 |
| Ensemble | ENSG00000047457 | ENSMUSG00000003617 |
| UniProt | P00450 | Q61147 |
| Refseq (mRNA) | NM_000096 | NM_001042611 |
| Refseq (protein) | NP_000087 | NP_001263177 |
| Gene locus | Chr 3: 149.16 - 149.22 Mb | Chr 3: 19.96 - 20.01 Mb |
| PubMed search | 1356 |
12870
|
Ceruloplasmin (CP) ( Latin caeruleus , blue '), in other spellings ceruloplasmin , ceruloplasmin or Zäruloplasmin , is a plasma protein of all new mouths . This bluish enzyme ( oxidase ) acts as a copper store and helps move iron out of cells where it has accumulated. It is formed in the liver and the brain and already receives six or seven copper ions post-translationally during its biosynthesis and can transport up to 8 copper ions per molecule. If there is a lack of copper in the body, on the other hand, only unstable apocoeruloplasmin is formed. Hereditary deficiency through mutation in the CP gene leads to pathological accumulations of iron in reticuloendothelial cells and hepatocytes .
Caeruloplasmin has a copper-dependent oxidase activity (ferroxidase), which enables the oxidation of Fe2 + to Fe3 + and thus contributes to its transport into the blood plasma in association with transferrin , which can only transport Fe3 + ions.
Serum ceruloplasmin is determined in the diagnosis of iron and copper metabolism. Serum levels can rise in severe infectious diseases (as part of the acute phase reaction ) and during pregnancy . In contrast, Wilson's disease (hepatolenticular degeneration) has decreased values below 0.2 g / l. If the ferroxidase activity is completely eliminated, which is associated with increased iron storage in the body, it is called acaeruloplasminemia .
Individual evidence
- ↑ Ceruloplasmin. In: Online Mendelian Inheritance in Man . (English).
- ↑ Nathan E. Hellman and Jonathan D. Gitlin: Ceruloplasmin metabolism and function. Annual Review of Nutrition Vol. 22: 439-458 doi : 10.1146 / annurev.nutr.22.012502.114457