Detail Information for IndEnz0002014270
IED ID IndEnz0002014270
Enzyme Type ID protease014270
Protein Name Complement factor H
H factor 1
Gene Name CFH HF HF1 HF2
Organism Homo sapiens (Human)
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 Primates Haplorrhini Simiiformes Catarrhini Hominoidea (apes) Hominidae (great apes) Homininae Homo Homo sapiens (Human)
Enzyme Sequence MRLLAKIICLMLWAICVAEDCNELPPRRNTEILTGSWSDQTYPEGTQAIYKCRPGYRSLGNVIMVCRKGEWVALNPLRKCQKRPCGHPGDTPFGTFTLTGGNVFEYGVKAVYTCNEGYQLLGEINYRECDTDGWTNDIPICEVVKCLPVTAPENGKIVSSAMEPDREYHFGQAVRFVCNSGYKIEGDEEMHCSDDGFWSKEKPKCVEISCKSPDVINGSPISQKIIYKENERFQYKCNMGYEYSERGDAVCTESGWRPLPSCEEKSCDNPYIPNGDYSPLRIKHRTGDEITYQCRNGFYPATRGNTAKCTSTGWIPAPRCTLKPCDYPDIKHGGLYHENMRRPYFPVAVGKYYSYYCDEHFETPSGSYWDHIHCTQDGWSPAVPCLRKCYFPYLENGYNQNYGRKFVQGKSIDVACHPGYALPKAQTTVTCMENGWSPTPRCIRVKTCSKSSIDIENGFISESQYTYALKEKAKYQCKLGYVTADGETSGSITCGKDGWSAQPTCIKSCDIPVFMNARTKNDFTWFKLNDTLDYECHDGYESNTGSTTGSIVCGYNGWSDLPICYERECELPKIDVHLVPDRKKDQYKVGEVLKFSCKPGFTIVGPNSVQCYHFGLSPDLPICKEQVQSCGPPPELLNGNVKEKTKEEYGHSEVVEYYCNPRFLMKGPNKIQCVDGEWTTLPVCIVEESTCGDIPELEHGWAQLSSPPYYYGDSVEFNCSESFTMIGHRSITCIHGVWTQLPQCVAIDKLKKCKSSNLIILEEHLKNKKEFDHNSNIRYRCRGKEGWIHTVCINGRWDPEVNCSMAQIQLCPPPPQIPNSHNMTTTLNYRDGEKVSVLCQENYLIQEGEEITCKDGRWQSIPLCVEKIPCSQPPQIEHGTINSSRSSQESYAHGTKLSYTCEGGFRISEENETTCYMGKWSSPPQCEGLPCKSPPEISHGVVAHMSDSYQYGEEVTYKCFEGFGIDGPAIAKCLGEKWSHPPSCIKTDCLSLPSFENAIPMGEKKDVYKAGEQVTYTCATYYKMDGASNVTCINSRWTGRPTCRDTSCVNPPTVQNAYIVSRQMSKYPSGERVRYQCRSPYEMFGDEEVMCLNGNWTEPPQCKDSTGKCGPPPPIDNGDITSFPLSVYAPASSVEYQCQNLYQLEGNKRITCRNGQWSEPPKCLHPCVISREIMENYNIALRWTAKQKLYSRTGESVEFVCKRGYRLSSRSHTLRTTCWDGKLEYPTCAKR
Enzyme Length 1231
Uniprot Accession Number P08603
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 (PubMed:21285368, PubMed:25402769). 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 (PubMed:19503104, PubMed:26700768). As a cofactor of the serine protease factor I, CFH also regulates proteolytic degradation of already-deposited C3b (PubMed:18252712, PubMed:28671664). 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 (PubMed:9558116, PubMed:20008295). {ECO:0000269|PubMed:18252712, ECO:0000269|PubMed:19503104, ECO:0000269|PubMed:20008295, ECO:0000269|PubMed:21285368, ECO:0000269|PubMed:25402769, ECO:0000269|PubMed:26700768, ECO:0000269|PubMed:28671664, ECO:0000269|PubMed:9558116}.
Temperature Dependency
PH Dependency
Pathway
nucleotide Binding
Features Alternative sequence (2); Beta strand (105); Chain (1); Disulfide bond (40); Domain (20); Frameshift (1); Glycosylation (9); Helix (6); Mutagenesis (7); Natural variant (59); Sequence conflict (4); Signal peptide (1); Turn (3)
Keywords 3D-structure;Age-related macular degeneration;Alternative splicing;Complement alternate pathway;Direct protein sequencing;Disease variant;Disulfide bond;Glycoprotein;Hemolytic uremic syndrome;Host-virus interaction;Immunity;Innate immunity;Reference proteome;Repeat;Secreted;Signal;Sulfation;Sushi
Interact With P02649; P01024; P01024; P01024; Itself; P02741; P02741; P26022; P26022; P02769; P16946; P13605; A0A024A2C9; Q9JXV4; Q932F7; P02741; P02741; P26022; P26022; P02741
Induction
Subcellular Location SUBCELLULAR LOCATION: Secreted.
Modified Residue
Post Translational Modification PTM: Sulfated on tyrosine residues. {ECO:0000269|PubMed:25136834}.
Signal Peptide SIGNAL 1..18; /evidence=ECO:0000269|PubMed:6215918
Structure 3D NMR spectroscopy (11); X-ray crystallography (33)
Cross Reference PDB 1HAQ; 1HCC; 1HFH; 1HFI; 2BZM; 2G7I; 2IC4; 2JGW; 2JGX; 2KMS; 2QFG; 2QFH; 2RLP; 2RLQ; 2UWN; 2V8E; 2W80; 2W81; 2WII; 2XQW; 3GAU; 3GAV; 3GAW; 3KXV; 3KZJ; 3OXU; 3R62; 3RJ3; 3SW0; 4AYD; 4AYE; 4AYI; 4AYM; 4B2R; 4B2S; 4J38; 4K12; 4ONT; 4ZH1; 5NBQ; 5O32; 5O35; 5WTB; 6ATG;
Mapped Pubmed ID 1067618; 10781834; 11399083; 11825898; 11921353; 12391176; 12424708; 12471127; 12630812; 14638802; 14974950; 15163532; 15174051; 15331938; 15557185; 15634279; 15749837; 15754282; 15784724; 16174643; 16192651; 16229850; 16263173; 16267773; 16299065; 16300415; 16310045; 16379025; 16386793; 16431947; 16470555; 16519819; 16528247; 16533809; 16541016; 16621965; 16630992; 16642439; 16710702; 16723442; 16730735; 16754848; 16774956; 16785547; 16787919; 16816528; 16828512; 16849663; 16865697; 16877387; 16885922; 16889549; 16905558; 16909242; 16936080; 16936129; 16936732; 16936733; 16954704; 17000705; 17003406; 17022693; 17076561; 17079491; 17137217; 17151483; 17157600; 17167412; 17198853; 17208302; 17210851; 17210852; 17210858; 17229916; 17241667; 17266113; 17285240; 17293598; 17306880; 17314151; 17339482; 17352366; 17362990; 17396242; 17398321; 17399790; 17426452; 17438519; 17438673; 17442969; 17456821; 17464302; 17472578; 17483111; 17558024; 17562771; 17580967; 17591627; 17591866; 17599974; 17631852; 17679948; 17697822; 17699195; 17724217; 17767156; 17846368; 17846371; 17869048; 17877809; 17915330; 17962488; 17973958; 17995985; 17999207; 18006700; 18039838; 18050121; 18054635; 18067970; 18093091; 18161619; 18162041; 18163432; 18164066; 18183578; 18203751; 18211923; 18223247; 18235016; 18235085; 18248681; 18263814; 18268093; 18292569; 18292760; 18316707; 18325906; 18336910; 18340363; 18362109; 18378209; 18406463; 18413232; 18421087; 18422436; 18423869; 18433936; 18436811; 18452766; 18461138; 18493315; 18502988; 18515590; 18538409; 18541031; 18566420; 18596911; 18604638; 18624398; 18627285; 18627465; 18658028; 18676680; 18682806; 18682812; 18704199; 18718667; 18762075; 18766990; 18784628; 18786923; 18787502; 18824548; 18852870; 18936151; 18939352; 18976665; 18979363; 18996904; 19000922; 19001225; 19003634; 19005013; 19009024; 19015224; 19019939; 19029036; 19043567; 19047406; 19048105; 19050261; 19074778; 19076828; 19092117; 19098018; 19117936; 19124749; 19144569; 19162324; 19166692; 19168221; 19169230; 19169232; 19170196; 19187590; 19187823; 19197355; 19202148; 19204088; 19242507; 19259132; 19299737; 19306911; 19308255; 19336370; 19336475; 19344414; 19351878; 19399715; 19428307; 19454698; 19505474; 19508377; 19531976; 19540598; 19541934; 19549636; 19567623; 19568762; 19625176; 19646183; 19680263; 19680273; 19692124; 19703145; 19704120; 19796758; 19797206; 19822851; 19822855; 19823576; 19825847; 19833879; 19845562; 19850343; 19850835; 19850925; 19861685; 19878322; 19899988; 19913121; 19948975; 19951950; 20007824; 20038862; 20042647; 20042653; 20059470; 20106822; 20127520; 20132989; 20157618; 20161521; 20163251; 20181037; 20237496; 20238042; 20299368; 20304497; 20346514; 20351616; 20378180; 20381870; 20385819; 20394361; 20513355; 20523265; 20534299; 20538655; 20574013; 20576771; 20580090; 20581873; 20588308; 20595690; 20605213; 20606025; 20622116; 20626361; 20628086; 20660596; 20664795; 20678803; 20688737; 20694013; 20703214; 20708732; 20711500; 20730046; 20734203; 20736788; 20801516; 20823202; 20841369; 20843825; 20854881; 20855886; 20861866; 20865640; 20865641; 20869121; 20878164; 20881291; 20888482; 21106043; 21111031; 21153520; 21158586; 21163532; 21167605; 21232084; 21270465; 21282580; 21317894; 21325619; 21339361; 21396937; 21397333; 21402993; 21435440; 21503575; 21531728; 21555552; 21558292; 21574022; 21618592; 21640790; 21642625; 21659506; 21677636; 21695352; 21697597; 21717289; 21743006; 21790300; 21793561; 21816153; 21817962; 21825189; 21856016; 21868097; 21871809; 21881555; 21882633; 21896867; 21930971; 21936007; 21979047; 21988832; 22022419; 22035603; 22059990; 22065918; 22065928; 22088229; 22104107; 22133792; 22136554; 22140259; 22153652; 22171659; 22182977; 22197220; 22253316; 22259222; 22293892; 22300950; 22302353; 22320225; 22426290; 22471560; 22486323; 22487577; 22491393; 22514678; 22526676; 22570352; 22628306; 22669321; 22786770; 22815489; 22840423; 22848687; 22875704; 22876110; 22894814; 22905135; 22922817; 22933840; 22936692; 22956584; 22965593; 22977134; 23070659; 23071278; 23098369; 23110143; 23111182; 23112567; 23195022; 23209669; 23233260; 23235303; 23243267; 23289807; 23289808; 23296223; 23300094; 23306536; 23332154; 23337555; 23356914; 23362846; 23365660; 23376722; 23385359; 23390291; 23414945; 23423485; 23441108; 23494043; 23497844; 23534868; 23562165; 23596508; 23637600; 23637608; 23660864; 23661701; 23662819; 23687431; 23701234; 23702979; 23747994; 23797962; 23844226; 23863906; 23864767; 23867343; 23867466; 23873044; 23880784; 23922956; 23938460; 23966370; 23972322; 23991205; 24008159; 24014239; 24080590; 24083687; 24113783; 24136289; 24221349; 24280833; 24333077; 24344133; 24362810; 24365176; 24440287; 24453474; 24498017; 24520367; 24534755; 24557084; 24586071; 24612979; 24632373; 24664701; 24692129; 24702844; 24722444; 24825900; 24835590; 24847905; 24863099; 24865190; 24885016; 24906858; 24953792; 24970616; 24974817; 24983375; 25030427; 25077532; 25091949; 25181963; 25188723; 25200399; 25205734; 25205864; 25277308; 25402348; 25439433; 25447048; 25612476; 25627090; 25628052; 25659429; 25695752; 25715554; 25733390; 25742403; 25771815; 25781901; 25803806; 25805734; 25814824; 25814826; 25880396; 25921341; 25925370; 25994214; 26035554; 26045838; 26132079; 26135246; 26152901; 26163426; 26181365; 26186240; 26215151; 26218915; 26243271; 26260032; 26268811; 26275133; 26296146; 26306739; 26312598; 26332911; 26354764; 26376859; 26399238; 26411831; 26439641; 26459349; 26468283; 26490391; 26490493; 26496610; 26501415; 26597206; 26614632; 26654980; 26703217; 26715202; 26727378; 26728463; 26733258; 26745149; 26746578; 26767664; 26768663; 26802141; 26826462; 26848857; 26852301; 26854823; 26938503; 26941266; 26942649; 26961257; 27035765; 27068115; 27099340; 27113631; 27116510; 27151934; 27196323; 27241480; 27262511; 27269047; 27316788; 27364548; 27393384; 27404493; 27419833; 27471039; 27502478; 27552409; 27572114; 27721145; 27778189; 27805046; 27832277; 27918307; 27939215; 28005184; 28011711; 28095095; 28105653; 28159936; 28403670; 28490660; 28551622; 28558370; 28593728; 28637873; 28641277; 28859202; 28941939; 29096998; 29240274; 29288229; 29311295; 29374201; 29377444; 29398083; 29410599; 29453225; 29579408; 29686068; 29720453; 29801032; 29885865; 29888403; 29912491; 29982679; 30013157; 30073298; 30139996; 301546; 30179527; 30217822; 30219152; 30253188; 30280493; 30285522; 30300269; 30320589; 30389424; 30519244; 30596689; 30630982; 30745371; 30804016; 30804422; 30808757; 30820144; 30838755; 30905644; 31130605; 31253657; 31331787; 31358387; 31525397; 31541546; 31658355; 31815877; 31861421; 31916060; 31925659; 31970928; 31984663; 32022122; 32138827; 32321835; 32328755; 32378251; 32406777; 32476814; 32507953; 32587311; 32838591; 32923173; 33178228; 33198211; 33309135; 33384694; 33387109; 33519811; 33536243; 33552059; 33643312; 33708358; 33732239; 33868311; 33887508; 33900362; 33918210; 33986750; 33995361; 34112227; 34139209; 34189567; 34233521; 34239032; 34260948; 34445430; 34556298; 34795662; 3848661; 6219696;
Motif
Gene Encoded By
Mass 139,096
Kinetics
Metal Binding
Rhea ID
Cross Reference Brenda