Protein family
The protein has not catalytic activity
Protein sequence
Protein function
Publications
Catalytic activity
| PDB | Resolution (Å) | PDB name |
|---|---|---|
| 1HZF | 2.3 | C4ADG FRAGMENT OF HUMAN COMPLEMENT FACTOR C4A |
| 5JPM | 3.75 | Structure of the complex of human complement C4 with MASP-2 rebuilt using iMDFF |
| 5JPN | 3.6 | Structure of human complement C4 rebuilt using iMDFF |
| 5JTW | 3.5 | Crystal structure of complement C4b re-refined using iMDFF |
| 6YSQ | 3.3 | The hC4Nb8 complement inhibitory nanobody in complex with C4b |
GO ontology
|
GO term | GO description |
|---|---|---|
| Biological Process | GO:0006956 | complement activation |
| Biological Process | GO:0006958 | complement activation, classical pathway |
| Biological Process | GO:0006954 | inflammatory response |
| Biological Process | GO:0045087 | innate immune response |
| Biological Process | GO:2000427 | positive regulation of apoptotic cell clearance |
| Biological Process | GO:0050778 | positive regulation of immune response |
| Biological Process | GO:1903028 | positive regulation of opsonization |
| Molecular Function | GO:0001849 | complement component C1q complex binding |
| Molecular Function | GO:0004866 | endopeptidase inhibitor activity |
| Cellular Component | GO:0030424 | axon |
| Cellular Component | GO:0072562 | blood microparticle |
| Cellular Component | GO:0005601 | classical-complement-pathway C3/C5 convertase complex |
| Cellular Component | GO:0030425 | dendrite |
| 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:0043025 | neuronal cell body |
| Cellular Component | GO:0005886 | plasma membrane |
| Cellular Component | GO:0045202 | synapse |
| InterPro
|
InterPro name |
|---|---|
| IPR000020 | Anaphylatoxin/fibulin |
| IPR001134 | Netrin domain |
| IPR001599 | Alpha-2-macroglobulin |
| IPR001840 | Anaphylatoxin, complement system domain |
| IPR002890 | Macroglobulin domain |
| IPR008930 | Terpenoid cyclases/protein prenyltransferase alpha-alpha toroid |
| IPR008993 | Tissue inhibitor of metalloproteinases-like, OB-fold |
| IPR009048 | Alpha-macroglobulin, receptor-binding |
| IPR011625 | Alpha-2-macroglobulin, bait region domain |
| IPR011626 | Alpha-macroglobulin-like, TED domain |
| IPR013783 | Immunoglobulin-like fold |
| IPR018081 | Anaphylatoxin, complement system |
| IPR018933 | Netrin module, non-TIMP type |
| IPR019742 | Alpha-2-macroglobulin, conserved site |
| IPR036595 | Alpha-macroglobulin, receptor-binding domain superfamily |
| IPR040839 | Macroglobulin domain MG4 |
| IPR041555 | Macroglobulin domain MG3 |
| IPR047565 | Alpha-macroglobulin-like, thiol-ester bond-forming region |
| Pfam
|
Pfam name |
|---|---|
| PF00207 | Alpha-2-macroglobulin family |
| PF01759 | UNC-6/NTR/C345C module |
| PF01821 | Anaphylotoxin-like domain |
| PF01835 | MG2 domain |
| PF07677 | A-macroglobulin receptor binding domain |
| PF07678 | A-macroglobulin TED domain |
| PF07703 | Alpha-2-macroglobulin bait region domain |
| PF17789 | Macroglobulin domain MG4 |
| PF17791 | Macroglobulin domain MG3 |
| Reactome
|
Reactome Name | Node type
|
Reactome Root | Reactome Root Name |
|---|---|---|---|---|
| R-HSA-166663 | Initial triggering of complement | Internal node | R-HSA-168256 | Immune System |
| R-HSA-174577 | Activation of C3 and C5 | Leaf | R-HSA-168256 | Immune System |
| 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-8957275 | Post-translational protein phosphorylation | Leaf | R-HSA-392499 | Metabolism of proteins |
| R-HSA-977606 | Regulation of Complement cascade | Leaf | R-HSA-168256 | Immune System |
| Location
|
ECO term
|
Pubmed |
|---|---|---|
| Cell projection, axon | ECO:0000269 | PubMed:26814963 |
| Cell projection, dendrite | ECO:0000269 | PubMed:26814963 |
| Secreted | ||
| Synapse | ECO:0000269 | PubMed:26814963 |
| HPO ID
|
HPO name |
|---|---|
| HP:0000007 | Autosomal recessive inheritance |
| HP:0000083 | Renal insufficiency |
| HP:0000099 | Glomerulonephritis |
| HP:0000155 | Oral ulcer |
| HP:0000488 | Retinopathy |
| HP:0000518 | Cataract |
| HP:0000613 | Photophobia |
| HP:0000618 | Blindness |
| HP:0000708 | Atypical behavior |
| HP:0000737 | Irritability |
| HP:0000979 | Purpura |
| HP:0000992 | Cutaneous photosensitivity |
| HP:0001061 | Acne |
| HP:0001097 | Keratoconjunctivitis sicca |
| HP:0001250 | Seizure |
| HP:0001251 | Ataxia |
| HP:0001269 | Hemiparesis |
| HP:0001287 | Meningitis |
| HP:0001288 | Gait disturbance |
| HP:0001289 | Confusion |
| HP:0001347 | Hyperreflexia |
| HP:0001369 | Arthritis |
| HP:0001482 | Subcutaneous nodule |
| HP:0001637 | Abnormal myocardium morphology |
| HP:0001653 | Mitral regurgitation |
| HP:0001658 | Myocardial infarction |
| HP:0001659 | Aortic regurgitation |
| HP:0001701 | Pericarditis |
| HP:0001733 | Pancreatitis |
| HP:0001744 | Splenomegaly |
| HP:0001824 | Weight loss |
| HP:0001945 | Fever |
| HP:0002017 | Nausea and vomiting |
| HP:0002024 | Malabsorption |
| HP:0002027 | Abdominal pain |
| HP:0002039 | Anorexia |
| HP:0002076 | Migraine |
| HP:0002102 | Pleuritis |
| HP:0002105 | Hemoptysis |
| HP:0002113 | Pulmonary infiltrates |
| HP:0002202 | Pleural effusion |
| HP:0002204 | Pulmonary embolism |
| HP:0002239 | Gastrointestinal hemorrhage |
| HP:0002321 | Vertigo |
| HP:0002354 | Memory impairment |
| HP:0002376 | Developmental regression |
| HP:0002383 | Infectious encephalitis |
| HP:0002516 | Increased intracranial pressure |
| HP:0002633 | Vasculitis |
| HP:0002637 | Cerebral ischemia |
| HP:0002716 | Lymphadenopathy |
| HP:0002725 | Systemic lupus erythematosus |
| HP:0002829 | Arthralgia |
| HP:0003326 | Myalgia |
| HP:0003401 | Paresthesia |
| HP:0004420 | Arterial thrombosis |
| HP:0004936 | Venous thrombosis |
| HP:0006824 | Cranial nerve paralysis |
| HP:0007256 | Abnormal pyramidal sign |
| HP:0008066 | Abnormal blistering of the skin |
| HP:0010885 | Avascular necrosis |
| HP:0011107 | Recurrent aphthous stomatitis |
| HP:0012378 | Fatigue |
| HP:0012649 | Increased inflammatory response |
| HP:0025434 | Reduced hemolytic complement activity |
| HP:0045042 | Decreased circulating complement C4 concentration |
| HP:0100326 | Immunologic hypersensitivity |
| HP:0100584 | Endocarditis |
| HP:0100614 | Myositis |
| HP:0100653 | Optic neuritis |
| HP:0100654 | Retrobulbar optic neuritis |
| HP:0100758 | Gangrene |
| HP:0100796 | Orchitis |
| HP:0100820 | Glomerulopathy |
| HP:0200034 | Papule |
| Tissue
|
Cell type
|
Expression level | Reliability |
|---|---|---|---|
| liver | hepatocytes | Low | Supported |
| placenta | cytotrophoblasts | Medium | Supported |
| placenta | endothelial cells | Medium | Supported |
| placenta | syncytiotrophoblasts - cell body | Medium | Supported |
| placenta | syncytiotrophoblasts - microvilli | High | Supported |
| Disease name | MONDO ID
|
ICD10
|
ICD10 chapter
|
OMIM
|
Orphanet
|
|---|---|---|---|---|---|
| systemic lupus erythematosus | MONDO:0007915 | M32 | chapter13, Diseases of the musculoskeletal system and connective tissue | OMIM:152700 | Orphanet:536 |
| complement component 4a deficiency | MONDO:0013721 | D84 | chapter3, Diseases of the blood and blood-forming organs and certain disorders involving the immune mechanism | OMIM:614380 |