NationMaster - Encyclopedia: Complement factor I

Complement Factor I (fI) is a protein of the Complement system, first isolated in 1966 in guinea pig serum^[1] that regulates complement activation by cleaving cell-bound or fluid phase C3b and C4b.^[2] Hugo is a masculine name. ... The Entrez logo The Entrez Global Query Cross-Database Search System allows access to databases at the National Center for Biotechnology Information (NCBI) website. ... The Mendelian Inheritance in Man project is a database that catalogues all the known diseases with a genetic component, and - when possible - links them to the relevant genes in the human genome. ... The National Center for Biotechnology Information (NCBI) is part of the US National Library of Medicine (NLM), which is a branch of the US National Institutes of Health. ... Swiss-Prot is a curated biological database of protein sequences created in 1986 by Amos Bairoch during his PhD and developed by the Swiss Institute of Bioinformatics and the European Bioinformatics Institute. ... The Enzyme Commission number (EC number) is a numerical classification scheme for enzymes, based on the chemical reactions they catalyze. ... Short and long arms Chromosome. ... Chromosome 4 is one of the 23 pairs of chromosomes in humans. ... A representation of the 3D structure of myoglobin, showing coloured alpha helices. ... A complement protein attacking an invader. ... Binomial name Cavia porcellus (Linnaeus, 1758) Guinea pigs (also called cavies) are rodents belonging to the family Caviidae and the genus Cavia, originally indigenous to the Andes. ... Blood plasma is the liquid component of blood, in which the blood cells are suspended. ...

Factor I deficiency in turn leads to low levels of complement component 3 (C3) in plasma, due to unregulated activation of the complement alternative pathway, and it has been associated with recurrent bacterial infections in children; more recently, mutations in the Factor I gene have been shown to be implicated ^[3] in development of Haemolytic Uremic Syndrome, a renal disease also caused by unregulated complement activation. In immunology, soluble C3-convertase, also known as iC3Bb, catalyzes the proteolytic cleavage of C3 into C3a and C3b as part of the alternative complement system. ... In medicine, Hemolytic-uremic syndrome (or haemolytic-uraemic syndrome, abbreviated HUS) is a disease characterised by microangiopathic hemolytic anemia, acute renal failure and a low platelet count (thrombocytopenia). ...

The gene for Factor I in humans is located on chromosome 4 ^[4] Factor I is synthesised mostly in the liver, and is initially secreted as a single 88 kDalton gene product; this precursor protein is then cleaved by furin to yield the mature fI protein, which is a disulphide-linked dimer of heavy chain (residues 19-335, 51 kDalton) and light chain (residues 340-583, 37 kDalton). Only the mature protein is active. Both heavy and light chains bear Asn-linked glycans, on three distinct glycosylation sites each. The fI heavy chain has four domains: a FIMAC domain, a Scavenger Receptor Cysteine Rich (SRCR) domain and two LDL-receptor Class A domains; the precise biological function of the heavy chain is not known, but it is likely to play a key role in recognising the fI cleavage substrates (C3b and C4b) and the cofactor proteins needed for cleavage of C3b (Factor H, CR1, MCP) and C4b (C4BP). The fI light chain is the serine protease domain containing the catalytic triad responsible for specific cleavage of C3b and C4b. For other meanings of this term, see gene (disambiguation). ... Chromosome 4 is one of the 23 pairs of chromosomes in humans. ... A protein precursor is an inactive protein (or peptide) that can be turned into an active form by posttranslational modification. ... Furin is a protease of animal cells that is similar in structure to the bacterial protease subtilisin. ... A disulfide bond (SS-bond), also called a disulfide bridge, is a strong covalent bond between two sulfhydryl groups. ... Sucrose, or common table sugar, is composed of glucose and fructose. ... Asparagine is one of the 20 most common natural amino acids on Earth. ... Glycans are polysaccharides. ... Glycosylation is the process or result of addition of saccharides to proteins and lipids. ... Within a protein, a structural domain (domain) is an element of overall structure that is self-stabilizing and often folds independently of the rest of the protein chain. ... In biochemistry, a substrate is a molecule upon which an enzyme acts. ... Crystal structure of Trypsin, a typical serine protease. ... Three amino acid residues found inside the active site of certain proteases. ...

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Proteins: complement system

Activators: C6 - C7 - C9 - MBL2 - PFC
Complexes: C1Q (C1QA, C1QB, C1QG) - C3-convertase - C8 (C8A, C8B, C8G) - MAC
Enzymes: BF - C1R - C1S - C2 - C3 - C4 - C5 - DF - MASP1 - MASP2
Inhibitors: C1inh - C4BP (C4BPA, C4BPB) - CLU - DAF - HF1 - IF - SERPING1 - VTN
Complement receptors: C3AR1 - C5R1 - CR1 - CR2 - ITGAM - CD46 A representation of the 3D structure of myoglobin, showing coloured alpha helices. ... A complement protein attacking an invader. ... Complement component 6 is a protein involved in the complement system. ... Complement component 7 is a protein involved in the complement system. ... Complement component 9 is a protein involved in the complement system. ... MBL2 is a protein involved in the complement system. ... PFC is a protein involved in the complement system. ... The C1q complex is potentially multivalent for attachment to the complement fixation sites of immunoglobulin. ... Complement component 1QA is a protein involved in the complement system. ... Complement component 1QB is a protein involved in the complement system. ... Complement component 1QG is a protein involved in the complement system. ... C3-convertase is the name of the enzyme composed of the C4b-C2b complex, which forms during the Classical pathyway of the complement system. ... Complement component 8A is a protein involved in the complement system. ... Complement component 8B is a protein involved in the complement system. ... Complement component 8G is a protein involved in the complement system. ... MAC is also called the killer molecule. ... BF is a protein involved in the complement system. ... Complement component 1R is a protein involved in the complement system. ... Complement component 1S is a protein involved in the complement system. ... Complement component 2 is a protein involved in the complement system. ... In immunology, soluble C3-convertase, also known as iC3Bb, catalyzes the proteolytic cleavage of C3 into C3a and C3b as part of the alternative complement system. ... Complement component 4 is a protein involved in the complement system. ... Complement component 5 is a protein involved in the complement system. ... DF is a protein involved in the complement system. ... MASP1 is a protein involved in the complement system. ... MASP2 is a protein involved in the complement system. ... C1-inhibitor (C1inh) is a serine protease inhibitor (serpin) protein, the main function of which is inhibition of the complement system. ... C4BP (Complement C4B-binding protein) can refer to: C4BPA (protein) C4BPB (protein) Category: ... C4BPA is a protein involved in the complement system. ... C4BPB is a protein involved in the complement system. ... CLU is a protein involved in the complement system. ... Decay accelerating factor is a protein involved in the complement system. ... HF1 is a protein involved in the complement system. ... SERPING1 is a protein involved in the complement system. ... Vitronectin is an abundant glycoprotein found in blood plasma and the extra cellular matrix. ... A Complement receptor is a receptor of the complement system, a part of the mediated innate immune system. ... C3AR1 is a protein involved in the complement system. ... C5R1 is a protein involved in the complement system. ... Complement receptor 1 Also known as CR1, CD35, C3b/C4b receptor and immune adherence receptor. ... Complement component receptor 2 is a protein involved in the complement system. ... Integrin alpha M is a protein involved in the complement system. ... CD46 is an inhibitory complement receptor. ...

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Endopeptidases: serine proteases/serine endopeptidases

Trypsin - Chymotrypsin - Elastase (Neutrophil, Pancreatic) - Enteropeptidase Peptidases (proteases [pronounced pro-tea-aces] and proteolytic enzymes are also commonly used) are enzymes which break peptide bonds of proteins. ... Crystal structure of Trypsin, a typical serine protease. ... Trypsin (EC 3. ... Chymotrypsin (bovine Î³ chymotrypsin: PDB 1AB9, EC 3. ... Protein Crystal Growth Porcine Elastase In molecular biology, elastase is an enzyme from the class of proteases (or better peptidases) that break down proteins. ... Neutrophil elastase (or leukocyte elastase) is a type of enzyme that acts as a platelet activator. ... Pancreatic elastase is a form of elastase formed in the pancreas. ... Enteropeptidase or enterokinase is an enzyme involved in human digestion. ...

Acrosin - Pronase - Proprotein convertases (1, 2) - Subtilisin/Furin Acrosin (EC 3. ... Pronase is a serine protease found in Streptomyces griseus. ... Proprotein convertases are enzymes which convert prohormones into hormones. ... Proprotein convertase 1, generally called prohormone convertase 1 or PC1, is the enzyme that begins the proteolytic cleavage of prohormones to their intermediate (or sometimes completely cleaved) forms. ... Proprotein convertase 2 (PC2) is a serine protease and proprotein convertase It is also referred to as prohormone convertase 2. PC2, like proprotein convertase 1 (PC1), is an enzyme responsible for the maturation of many neuroendocrine peptides from their precursors, such as the conversion of proinsulin to insulin. ... Subtilisin is a proteolytic enzyme obtained from . ... Furin is a protease of animal cells that is similar in structure to the bacterial protease subtilisin. ...

Immune (Chymase, Granzyme, Tryptase, Proteinase 3/Myeloblastin) - Venombin (Ancrod, Batroxobin) Chymase is a serine protease found primarily in mast cells. ... Granzymes are exogenous serine proteases that are released by cytoplasmic granules within cytotoxic T cells and natural killer cells. ... The most abundant secretory granule-derived serine proteinase contained in mast cells that has recently been used as a marker for mast cell activation. ... Proteinase 3 is a serine protease enzyme expressed mainly in neutrophil granulocytes. ... // Basic Chemical, Pharmacological, and Marketing Information Ancrod (also known under its former brand name ArwinÂ® and recently ViprinexÂ®) is an anticoagulant with a triple mode of action. ... Batroxobin is a serine protease derived from the venom of Bothrops atrox. ...

Complement system: Factor B - Factor D - Factor I - MASP (MASP1, MASP2) BF is a protein involved in the complement system. ... DF is a protein involved in the complement system. ... Mannose-binding protein-associated serine protease are serine proteases involved in the complement system. ... MASP1 is a protein involved in the complement system. ... MASP2 is a protein involved in the complement system. ...

Coagulation factors: Thrombin - Factor VIIa - Factor IXa - Factor Xa - Factor XIa - Factor XIIa - Kallikrein (PSA) - Fibrinolysis: Plasmin - Tissue plasminogen activator - Urinary plasminogen activator Thrombin (activated Factor II) is a coagulation protein that has many effects in the coagulation cascade. ... Factor VII (old name proconvertin) is one of the central proteins in the coagulation cascade. ... Factor IX (or Christmas factor or Christmas-Eve factor) is one of the serine proteases (EC 3. ... Factor X, also known by the eponym Stuart-Prower factor or as thrombokinase, is an enzyme ( EC 3. ... Factor XI or plasma thromboplastin antecent is one of the enzymes ( EC 3. ... Hageman factor is a plasma protein now usually known as factor XII. It is part of the coagulation cascade and activates factor XI and prekallikrein. ... Kallikrien (KLK) enzymes are a group of serine proteases found in many different tissues and body fluids. ... Prostate specific antigen (PSA, also known as kallikrein III, seminin, semenogelase, Î³-seminoprotein and P-30 antigen) is a protein manufactured almost exclusively by the prostate gland; PSA is produced for the ejaculate where it liquifies the semen and allows sperm to swim freely. ... Plasmin is an important degrading enzyme (EC 3. ... In blood coagulation, tissue plasminogen activator (tPA) is an enzyme (EC 3. ... Urokinase, also called urokinase-type Plasminogen Activator (uPA) is an enzyme (EC 3. ...

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