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Ceruloplasmin (ferroxidase)
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| PDB rendering based on 1kcw. The Protein Data Bank ( PDB) is a repository for 3-D structural data of Proteins and Nucleic acids These data typically obtained by X-ray crystallography | ||
| Available structures: 1kcw, 2j5w | ||
| Identifiers | ||
| Symbol(s) | CP; CP-2 | |
| External IDs | OMIM: 117700 MGI: 88476 HomoloGene: 75 | |
| RNA expression pattern | ||
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| Orthologs | ||
| Human | Mouse | |
| Entrez | 1356 | 12870 |
| Ensembl | ENSG00000047457 | ENSMUSG00000003617 |
| Uniprot | P00450 | Q2F3J4 |
| Refseq | NM_000096 (mRNA) NP_000087 (protein) |
NM_001042611 (mRNA) NP_001036076 (protein) |
| Location | Chr 3: 150.37 - 150.42 Mb | Chr 3: 20.15 - 20.2 Mb |
| Pubmed search | [1] | [2] |
Ceruloplasmin (or caeruloplasmin) is officially known as ferroxidase or iron(II):oxygen oxidoreductase. The Human Genome Organisation (HUGO is an organization involved in the Human Genome Project, a project about mapping the human genome The Mouse Genome Informatics (MGI website is run by The Jackson Laboratory. HomoloGene, a tool of the National Center for Biotechnology Information (NCBI is a system for automated detection of homologs (similarity attributable to descent The Entrez Global Query Cross-Database Search System is a powerful Federated search engine or Web portal that allows users to search many discrete Health sciences Ensembl is a joint scientific project between the European Bioinformatics Institute and the Wellcome Trust Sanger Institute, which was launched in 1999 in response to the imminent UniProt is the uni versal prot ein resource a central repository of Protein data created by combining Swiss-Prot, TrEMBL PubMed is a free search engine for accessing the MEDLINE database of citations and abstracts of biomedical research articles It is the major copper-carrying protein in the blood, and in addition plays a role in iron metabolism. Copper (ˈkɒpɚ is a Chemical element with the symbol Cu (cuprum and Atomic number 29 Human iron metabolism is the set of chemical reactions maintaining Human homeostasis of Iron. It was first described in 1948. [1]
Contents |
Function
It is an enzyme (EC 1.16.3.1) synthesized in the liver containing 6 atoms of copper in its structure. Enzymes are Biomolecules that catalyze ( ie increase the rates of Chemical reactions Almost all enzymes are Proteins This article is about the Enzyme Commission codes For the European Commission system for coding chemicals see EC-No. Copper (ˈkɒpɚ is a Chemical element with the symbol Cu (cuprum and Atomic number 29 Ceruloplasmin carries 90% of the copper in our plasma. The other 10% is carried by albumin, albumin may be confused at times to have a greater importance as a copper carrier because it binds copper less tightly than ceruloplasmin. Albumin (Latin albus white refers generally to any Protein with water Solubility, which is moderately soluble in concentrated salt solutions and experiences Ceruloplasmin exhibits a copper-dependent oxidase activity, which is associated with possible oxidation of Fe2+ (ferrous iron) into Fe3+ (ferric iron), therefore assisting in its transport in the plasma in association with transferrin, which can only carry iron in the ferric state. Transferrin is a Blood plasma Protein for Iron Ion delivery Transferrin is a Glycoprotein, which binds iron very tightly but reversibly
Pathology
Like any other plasma protein, levels drop in patients with hepatic disease due to reduced synthesizing capabilities.
Mutations in the ceruloplasmin gene can lead to the rare genetic human disease aceruloplasminemia, characterized by iron overload in the brain, liver, pancreas, and retina. Aceruloplasminemia is an Autosomal Recessive disorder of Iron Metabolism characterized by progressive Neurodegeneration of the
Interpretation
Decreased levels
Lower-than-normal ceruloplasmin levels may indicate:
- Menkes disease (Menke's kinky hair syndrome) (very rare)
- Wilson's disease (a rare copper storage disease)[2]
- Overdose of vitamin C
- Copper deficiency
- Aceruloplasminemia[3]
Elevated levels
Greater-than-normal ceruloplasmin levels may indicate:
- pregnancy
- lymphoma
- acute and chronic inflammation (it is an acute-phase reactant)
- rheumatoid arthritis
References
- ^ Holmberg CG, Laurell C-B (1948). Menkes disease (also called the kinky hair disease or Menkes kinky hair syndrome) is a disorder that affects Copper levels in the body Wilson's disease or hepatolenticular degeneration is an Autosomal recessive Genetic disorder in which Copper accumulates in tissues Vitamin C or L-ascorbate is an Essential nutrient for a large number of higher primate species a small number of other Mammalian Copper (ˈkɒpɚ is a Chemical element with the symbol Cu (cuprum and Atomic number 29 Aceruloplasminemia is an Autosomal Recessive disorder of Iron Metabolism characterized by progressive Neurodegeneration of the Pregnancy ( Latin graviditas) is the carrying of one or more offspring known as a Fetus or Embryo, inside the Uterus of a Female Lymphoma a type of Neoplasm that originates in Lymphocytes (a type of White blood cell in the vertebrate Immune system) Inflammation ( Latin, inflamatio, to set on fire is the complex biological response of vascular tissues to harmful stimuli such as Pathogens Acute-phase proteins are a class of Proteins whose plasma concentrations increase (positive acute phase proteins or decrease (negative acute phase proteins in response to Rheumatoid arthritis ( RA) is a chronic systemic autoimmune disorder that causes the Immune system to attack the Joints, where "Investigations in serum copper. II. Isolation of the Copper containing protein, and a description of its properties". Acta Chem Scand 2: 550-56.
- ^ Scheinberg IH, Gitlin D (Oct 1952). "Deficiency of ceruloplasmin in patients with hepatolenticular degeneration (Wilson's disease)". Science 116 (3018): 484-5. doi:. A digital object identifier ( DOI) is a permanent identifier given to an Electronic document. PMID 12994898.
- ^ Gitlin JD (1998). "Aceruloplasminemia. ". Pediatr. Res. 44 (3): 271-6. doi:. A digital object identifier ( DOI) is a permanent identifier given to an Electronic document. PMID 9727700.
Further reading
- Hellman NE, Gitlin JD (2002). "Ceruloplasmin metabolism and function. ". Annu. Rev. Nutr. 22: 439-58. doi:. A digital object identifier ( DOI) is a permanent identifier given to an Electronic document. PMID 12055353.
- Mazumder B, Seshadri V, Fox PL (2003). "Translational control by the 3'-UTR: the ends specify the means. ". Trends Biochem. Sci. 28 (2): 91-8. doi:. A digital object identifier ( DOI) is a permanent identifier given to an Electronic document. PMID 12575997.
- Giurgea N, Constantinescu MI, Stanciu R, et al. (2005). "Ceruloplasmin - acute-phase reactant or endogenous antioxidant? The case of cardiovascular disease. ". Med. Sci. Monit. 11 (2): RA48-51. PMID 15668644.
- Kingston IB, Kingston BL, Putnam FW (1978). "Chemical evidence that proteolytic cleavage causes the heterogeneity present in human ceruloplasmin preparations. ". Proc. Natl. Acad. Sci. U. S. A. 74 (12): 5377-81. PMID 146197.
- Polosatov MV, Klimov PK, Masevich CG, et al. (1979). "Interaction of synthetic human big gastrin with blood proteins of man and animals. ". Acta hepato-gastroenterologica 26 (2): 154-9. PMID 463490.
- Schilsky ML, Stockert RJ, Pollard JW (1993). "Caeruloplasmin biosynthesis by the human uterus. ". Biochem. J. 288 ( Pt 2): 657-61. PMID 1463466.
- Walker FJ, Fay PJ (1990). "Characterization of an interaction between protein C and ceruloplasmin. ". J. Biol. Chem. 265 (4): 1834-6. PMID 2105310.
- Fleming RE, Gitlin JD (1990). "Primary structure of rat ceruloplasmin and analysis of tissue-specific gene expression during development. ". J. Biol. Chem. 265 (13): 7701-7. PMID 2332446.
- Yang FM, Friedrichs WE, Cupples RL, et al. (1990). "Human ceruloplasmin. Tissue-specific expression of transcripts produced by alternative splicing. ". J. Biol. Chem. 265 (18): 10780-5. PMID 2355023.
- Koschinsky ML, Funk WD, van Oost BA, MacGillivray RT (1986). "Complete cDNA sequence of human preceruloplasmin. ". Proc. Natl. Acad. Sci. U. S. A. 83 (14): 5086-90. doi:. A digital object identifier ( DOI) is a permanent identifier given to an Electronic document. PMID 2873574.
- Royle NJ, Irwin DM, Koschinsky ML, et al. (1987). "Human genes encoding prothrombin and ceruloplasmin map to 11p11-q12 and 3q21-24, respectively. ". Somat. Cell Mol. Genet. 13 (3): 285-92. doi:. A digital object identifier ( DOI) is a permanent identifier given to an Electronic document. PMID 3474786.
- Yang F, Naylor SL, Lum JB, et al. (1986). "Characterization, mapping, and expression of the human ceruloplasmin gene. ". Proc. Natl. Acad. Sci. U. S. A. 83 (10): 3257-61. doi:. A digital object identifier ( DOI) is a permanent identifier given to an Electronic document. PMID 3486416.
- Mercer JF, Grimes A (1986). "Isolation of a human ceruloplasmin cDNA clone that includes the N-terminal leader sequence. ". FEBS Lett. 203 (2): 185-90. doi:. A digital object identifier ( DOI) is a permanent identifier given to an Electronic document. PMID 3755405.
- Rask L, Valtersson C, Anundi H, et al. (1983). "Subcellular localization in normal and vitamin A-deficient rat liver of vitamin A serum transport proteins, albumin, ceruloplasmin and class I major histocompatibility antigens. ". Exp. Cell Res. 143 (1): 91-102. doi:. A digital object identifier ( DOI) is a permanent identifier given to an Electronic document. PMID 6337857.
- Kressner MS, Stockert RJ, Morell AG, Sternlieb I (1984). "Origins of biliary copper. ". Hepatology 4 (5): 867-70. PMID 6479854.
- Takahashi N, Bauman RA, Ortel TL, et al. (1983). "Internal triplication in the structure of human ceruloplasmin. ". Proc. Natl. Acad. Sci. U. S. A. 80 (1): 115-9. doi:. A digital object identifier ( DOI) is a permanent identifier given to an Electronic document. PMID 6571985.
- Takahashi N, Ortel TL, Putnam FW (1984). "Single-chain structure of human ceruloplasmin: the complete amino acid sequence of the whole molecule. ". Proc. Natl. Acad. Sci. U. S. A. 81 (2): 390-4. doi:. A digital object identifier ( DOI) is a permanent identifier given to an Electronic document. PMID 6582496.
- Dwulet FE, Putnam FW (1981). "Complete amino acid sequence of a 50,000-dalton fragment of human ceruloplasmin. ". Proc. Natl. Acad. Sci. U. S. A. 78 (2): 790-4. doi:. A digital object identifier ( DOI) is a permanent identifier given to an Electronic document. PMID 6940148.
- Kingston IB, Kingston BL, Putnam FW (1980). "Primary structure of a histidine-rich proteolytic fragment of human ceruloplasmin. I. Amino acid sequence of the cyanogen bromide peptides. ". J. Biol. Chem. 255 (7): 2878-85. PMID 6987229.
Dictionary
ceruloplasmin
-noun
- (biochemistry) A blue protein that contains copper; it acts as a ferroxidase in association with transferrin
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