Protein family
Protein sequence
Protein function
Catalytic activity
EC number
EC number description
1.16.3.1
Oxidoreductases;
Oxidizing metal ions;
With oxygen as acceptor;
ferroxidase
PDB | Resolution (Å) | PDB name |
---|---|---|
1KCW | 3.0 | X-RAY CRYSTAL STRUCTURE OF HUMAN CERULOPLASMIN AT 3.0 ANGSTROMS |
2J5W | 2.8 | Ceruloplasmin revisited: structural and functional roles of various metal cation binding sites |
4EJX | 4.69 | Structure of ceruloplasmin-myeloperoxidase complex |
4ENZ | 2.6 | Structure of human ceruloplasmin at 2.6 A resolution |
GO ontology | GO term | GO description |
---|---|---|
Biological Process | GO:0006825 | copper ion transport |
Biological Process | GO:0006879 | intracellular iron ion homeostasis |
Biological Process | GO:0006826 | iron ion transport |
Molecular Function | GO:0005507 | copper ion binding |
Molecular Function | GO:0004322 | ferroxidase activity |
Molecular Function | GO:0016491 | oxidoreductase activity |
Molecular Function | GO:0051087 | protein-folding chaperone binding |
Cellular Component | GO:0072562 | blood microparticle |
Cellular Component | GO:0005788 | endoplasmic reticulum lumen |
Cellular Component | GO:0070062 | extracellular exosome |
Cellular Component | GO:0005576 | extracellular region |
Cellular Component | GO:0005615 | extracellular space |
Cellular Component | GO:0005765 | lysosomal membrane |
Cellular Component | GO:0005886 | plasma membrane |
InterPro | InterPro name |
---|---|
IPR001117 | Multicopper oxidase, second cupredoxin domain |
IPR002355 | Multicopper oxidase, copper-binding site |
IPR008972 | Cupredoxin |
IPR011706 | Multicopper oxidase, C-terminal |
IPR011707 | Multicopper oxidase, N-terminal |
IPR033138 | Multicopper oxidases, conserved site |
IPR045087 | Multicopper oxidase |
Pfam | Pfam name |
---|---|
PF00394 | Multicopper oxidase |
PF07731 | Multicopper oxidase |
PF07732 | Multicopper oxidase |
Reactome | Reactome Name | Node type | Reactome Root | Reactome Root Name |
---|---|---|---|---|
R-HSA-381426 | Regulation of Insulin-like Growth Factor (IGF) transport and uptake by Insulin-like Growth Factor Binding Proteins (IGFBPs) | Leaf | R-HSA-392499 | Metabolism of proteins |
R-HSA-425410 | Metal ion SLC transporters | Internal node | R-HSA-382551 | Transport of small molecules |
R-HSA-5619049 | Defective SLC40A1 causes hemochromatosis 4 (HFE4) (macrophages) | Leaf | R-HSA-1643685 | Disease |
R-HSA-5619060 | Defective CP causes aceruloplasminemia (ACERULOP) | Leaf | R-HSA-1643685 | Disease |
R-HSA-8957275 | Post-translational protein phosphorylation | Leaf | R-HSA-392499 | Metabolism of proteins |
R-HSA-917937 | Iron uptake and transport | Internal node | R-HSA-382551 | Transport of small molecules |
Location | ECO term | Pubmed |
---|---|---|
Secreted | ECO:0000250 |
HPO ID | HPO name |
---|---|
HP:0000007 | Autosomal recessive inheritance |
HP:0000273 | Facial grimacing |
HP:0000473 | Torticollis |
HP:0000546 | Retinal degeneration |
HP:0000608 | Macular degeneration |
HP:0000639 | Nystagmus |
HP:0000643 | Blepharospasm |
HP:0000707 | Abnormality of the nervous system |
HP:0000726 | Dementia |
HP:0000741 | Apathy |
HP:0000819 | Diabetes mellitus |
HP:0001251 | Ataxia |
HP:0001260 | Dysarthria |
HP:0001300 | Parkinsonism |
HP:0001332 | Dystonia |
HP:0001337 | Tremor |
HP:0001394 | Cirrhosis |
HP:0001395 | Hepatic fibrosis |
HP:0001635 | Congestive heart failure |
HP:0001903 | Anemia |
HP:0002063 | Rigidity |
HP:0002066 | Gait ataxia |
HP:0002070 | Limb ataxia |
HP:0002071 | Abnormality of extrapyramidal motor function |
HP:0002072 | Chorea |
HP:0002168 | Scanning speech |
HP:0002304 | Akinesia |
HP:0002354 | Memory impairment |
HP:0002396 | Cogwheel rigidity |
HP:0003281 | Increased circulating ferritin concentration |
HP:0003581 | Adult onset |
HP:0004305 | Involuntary movements |
HP:0004840 | Hypochromic microcytic anemia |
HP:0005505 | Refractory anemia |
HP:0007703 | Abnormality of retinal pigmentation |
HP:0010837 | Decreased circulating ceruloplasmin concentration |
HP:0010994 | Abnormal corpus striatum morphology |
HP:0011967 | Decreased circulating copper concentration |
HP:0012090 | Abnormal pancreas morphology |
HP:0012179 | Craniofacial dystonia |
HP:0012379 | Abnormal circulating enzyme concentration or activity |
HP:0012465 | Elevated hepatic iron concentration |
HP:0012675 | Iron accumulation in brain |
HP:0012696 | Abnormal thalamic MRI signal intensity |
HP:0025498 | Aceruloplasminemia |
HP:0040303 | Decreased serum iron |
HP:0100321 | Abnormal dentate nucleus morphology |
HP:0100543 | Cognitive impairment |
Disease name | MONDO ID | ICD10 | ICD10 chapter | OMIM | Orphanet |
---|---|---|---|---|---|
aceruloplasminemia | MONDO:0011426 | G23 | chapter6, Diseases of the nervous system | OMIM:604290 | Orphanet:48818 |