Protein family
The protein has not catalytic activity
Protein sequence
Protein function
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 |
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 |