| IED ID | IndEnz0002004601 |
| Enzyme Type ID | protease004601 |
| Protein Name |
Complement factor H Protein beta-1-H |
| Gene Name | Cfh Hf1 |
| Organism | Mus musculus (Mouse) |
| Taxonomic Lineage | cellular organisms Eukaryota Opisthokonta Metazoa Eumetazoa Bilateria Deuterostomia Chordata Craniata Vertebrata Gnathostomata (jawed vertebrates) Teleostomi Euteleostomi Sarcopterygii Dipnotetrapodomorpha Tetrapoda Amniota Mammalia Theria Eutheria Boreoeutheria Euarchontoglires Glires (Rodents and rabbits) Rodentia Myomorpha (mice and others) Muroidea Muridae Murinae Mus Mus Mus musculus (Mouse) |
| Enzyme Sequence | MRLSARIIWLILWTVCAAEDCKGPPPRENSEILSGSWSEQLYPEGTQATYKCRPGYRTLGTIVKVCKNGKWVASNPSRICRKKPCGHPGDTPFGSFRLAVGSQFEFGAKVVYTCDDGYQLLGEIDYRECGADGWINDIPLCEVVKCLPVTELENGRIVSGAAETDQEYYFGQVVRFECNSGFKIEGHKEIHCSENGLWSNEKPRCVEILCTPPRVENGDGINVKPVYKENERYHYKCKHGYVPKERGDAVCTGSGWSSQPFCEEKRCSPPYILNGIYTPHRIIHRSDDEIRYECNYGFYPVTGSTVSKCTPTGWIPVPRCTLKPCEFPQFKYGRLYYEESLRPNFPVSIGNKYSYKCDNGFSPPSGYSWDYLRCTAQGWEPEVPCVRKCVFHYVENGDSAYWEKVYVQGQSLKVQCYNGYSLQNGQDTMTCTENGWSPPPKCIRIKTCSASDIHIDNGFLSESSSIYALNRETSYRCKQGYVTNTGEISGSITCLQNGWSPQPSCIKSCDMPVFENSITKNTRTWFKLNDKLDYECLVGFENEYKHTKGSITCTYYGWSDTPSCYERECSVPTLDRKLVVSPRKEKYRVGDLLEFSCHSGHRVGPDSVQCYHFGWSPGFPTCKGQVASCAPPLEILNGEINGAKKVEYSHGEVVKYDCKPRFLLKGPNKIQCVDGNWTTLPVCIEEERTCGDIPELEHGSAKCSVPPYHHGDSVEFICEENFTMIGHGSVSCISGKWTQLPKCVATDQLEKCRVLKSTGIEAIKPKLTEFTHNSTMDYKCRDKQEYERSICINGKWDPEPNCTSKTSCPPPPQIPNTQVIETTVKYLDGEKLSVLCQDNYLTQDSEEMVCKDGRWQSLPRCIEKIPCSQPPTIEHGSINLPRSSEERRDSIESSSHEHGTTFSYVCDDGFRIPEENRITCYMGKWSTPPRCVGLPCGPPPSIPLGTVSLELESYQHGEEVTYHCSTGFGIDGPAFIICEGGKWSDPPKCIKTDCDVLPTVKNAIIRGKSKKSYRTGEQVTFRCQSPYQMNGSDTVTCVNSRWIGQPVCKDNSCVDPPHVPNATIVTRTKNKYLHGDRVRYECNKPLELFGQVEVMCENGIWTEKPKCRDSTGKCGPPPPIDNGDITSLSLPVYEPLSSVEYQCQKYYLLKGKKTITCRNGKWSEPPTCLHACVIPENIMESHNIILKWRHTEKIYSHSGEDIEFGCKYGYYKARDSPPFRTKCINGTINYPTCV |
| Enzyme Length | 1234 |
| Uniprot Accession Number | P06909 |
| Absorption | |
| Active Site | |
| Activity Regulation | |
| Binding Site | |
| Calcium Binding | |
| catalytic Activity | |
| DNA Binding | |
| EC Number | |
| Enzyme Function | FUNCTION: Glycoprotein that plays an essential role in maintaining a well-balanced immune response by modulating complement activation. Acts as a soluble inhibitor of complement, where its binding to self markers such as glycan structures prevents complement activation and amplification on cell surfaces. Accelerates the decay of the complement alternative pathway (AP) C3 convertase C3bBb, thus preventing local formation of more C3b, the central player of the complement amplification loop. As a cofactor of the serine protease factor I, CFH also regulates proteolytic degradation of already-deposited C3b. In addition, mediates several cellular responses through interaction with specific receptors. For example, interacts with CR3/ITGAM receptor and thereby mediates the adhesion of human neutrophils to different pathogens. In turn, these pathogens are phagocytosed and destroyed. {ECO:0000250|UniProtKB:P08603}. |
| Temperature Dependency | |
| PH Dependency | |
| Pathway | |
| nucleotide Binding | |
| Features | Beta strand (10); Chain (1); Compositional bias (1); Disulfide bond (40); Domain (20); Glycosylation (7); Helix (2); Modified residue (1); Region (1); Sequence conflict (1); Signal peptide (1) |
| Keywords | 3D-structure;Complement alternate pathway;Disulfide bond;Glycoprotein;Immunity;Innate immunity;Phosphoprotein;Reference proteome;Repeat;Secreted;Signal;Sulfation;Sushi |
| Interact With | |
| Induction | |
| Subcellular Location | SUBCELLULAR LOCATION: Secreted {ECO:0000250|UniProtKB:P08603}. |
| Modified Residue | MOD_RES 1198; /note="Phosphoserine"; /evidence="ECO:0007744|PubMed:17242355, ECO:0007744|PubMed:21183079" |
| Post Translational Modification | PTM: Sulfated on tyrosine residues. {ECO:0000250|UniProtKB:P08603}. |
| Signal Peptide | SIGNAL 1..18; /evidence=ECO:0000250 |
| Structure 3D | X-ray crystallography (1) |
| Cross Reference PDB | 2YBY; |
| Mapped Pubmed ID | 10693874; 11217851; 11489951; 11739537; 12091909; 12466851; 12914820; 1311971; 1486806; 1495973; 15574507; 15582152; 16023208; 16597679; 16602821; 16769899; 16829211; 16849499; 1689298; 16936129; 17028856; 17410102; 17420242; 17517971; 17562771; 17921253; 18202746; 1834169; 18554416; 18947875; 19299737; 19411110; 19458267; 19541934; 19620346; 19828624; 20445496; 20538999; 20675597; 20702729; 2071146; 20813971; 20854881; 20962256; 21148254; 21148255; 21267068; 21302086; 21544060; 2165966; 21677750; 21862448; 21920606; 21979047; 22174452; 22219359; 22819137; 22832515; 22875704; 22918646; 23029250; 23184055; 23204401; 23436934; 23469078; 23487775; 23747511; 23799019; 23844226; 24006456; 24279761; 24370827; 24550112; 24664701; 24929970; 2522412; 25284781; 25447048; 25565310; 2564419; 25684976; 2574151; 25921262; 25991857; 26030099; 26149919; 26374608; 26768663; 26802141; 27165610; 27397653; 27564415; 27918307; 28057640; 28228282; 28535371; 2878046; 2892757; 2895697; 29070671; 29389016; 29392311; 29858280; 2993224; 30642982; 30705315; 30711487; 30808757; 31133737; 31354740; 31409673; 3156187; 32165615; 32369457; 32376801; 32737203; 32849636; 32962822; 33410734; 33558624; 33708358; 3384439; 33859044; 34054871; 34139209; 3416634; 4955525; 4992636; 540002; 6308669; 6332054; 6934364; 6967858; 6973596; 7165695; 7410386; 7474114; 7558020; 7894156; 8075500; 8422330; 8468048; 8482578; 8615824; 9799332; |
| Motif | |
| Gene Encoded By | |
| Mass | 139,138 |
| Kinetics | |
| Metal Binding | |
| Rhea ID | |
| Cross Reference Brenda |