IED Industry Enzyme Database

Detail Information for IndEnz0002013953
IED ID IndEnz0002013953
Enzyme Type ID protease013953
Protein Name Alpha-1-antitrypsin
Alpha-1 protease inhibitor
Alpha-1-antiproteinase
Serpin A1

Cleaved into: Short peptide from AAT
SPAAT
Gene Name SERPINA1 AAT PI PRO0684 PRO2209
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 MPSSVSWGILLLAGLCCLVPVSLAEDPQGDAAQKTDTSHHDQDHPTFNKITPNLAEFAFSLYRQLAHQSNSTNIFFSPVSIATAFAMLSLGTKADTHDEILEGLNFNLTEIPEAQIHEGFQELLRTLNQPDSQLQLTTGNGLFLSEGLKLVDKFLEDVKKLYHSEAFTVNFGDTEEAKKQINDYVEKGTQGKIVDLVKELDRDTVFALVNYIFFKGKWERPFEVKDTEEEDFHVDQVTTVKVPMMKRLGMFNIQHCKKLSSWVLLMKYLGNATAIFFLPDEGKLQHLENELTHDIITKFLENEDRRSASLHLPKLSITGTYDLKSVLGQLGITKVFSNGADLSGVTEEAPLKLSKAVHKAVLTIDEKGTEAAGAMFLEAIPMSIPPEVKFNKPFVFLMIEQNTKSPLFMGKVVNPTQK
Enzyme Length 418
Uniprot Accession Number P01009
Absorption
Active Site
Activity Regulation
Binding Site
Calcium Binding
catalytic Activity
DNA Binding
EC Number
Enzyme Function FUNCTION: Inhibitor of serine proteases. Its primary target is elastase, but it also has a moderate affinity for plasmin and thrombin. Irreversibly inhibits trypsin, chymotrypsin and plasminogen activator. The aberrant form inhibits insulin-induced NO synthesis in platelets, decreases coagulation time and has proteolytic activity against insulin and plasmin.; FUNCTION: [Short peptide from AAT]: Reversible chymotrypsin inhibitor. It also inhibits elastase, but not trypsin. Its major physiological function is the protection of the lower respiratory tract against proteolytic destruction by human leukocyte elastase (HLE).
Temperature Dependency
PH Dependency
Pathway
nucleotide Binding
Features Alternative sequence (2); Beta strand (19); Chain (1); Erroneous initiation (2); Glycosylation (3); Helix (11); Modified residue (3); Mutagenesis (1); Natural variant (38); Peptide (1); Region (1); Sequence conflict (14); Signal peptide (1); Site (3); Turn (7)
Keywords 3D-structure;Acute phase;Alternative splicing;Blood coagulation;Direct protein sequencing;Endoplasmic reticulum;Extracellular matrix;Glycoprotein;Hemostasis;Phosphoprotein;Protease inhibitor;Reference proteome;Secreted;Serine protease inhibitor;Signal
Interact With Q8N7X4; Itself; P43307; O15393; P00772; P71213; P00760
Induction
Subcellular Location SUBCELLULAR LOCATION: Secreted. Endoplasmic reticulum. Note=The S and Z allele are not secreted effectively and accumulate intracellularly in the endoplasmic reticulum.; SUBCELLULAR LOCATION: [Short peptide from AAT]: Secreted, extracellular space, extracellular matrix.
Modified Residue MOD_RES 38; /note=Phosphoserine; by FAM20C; /evidence=ECO:0000269|PubMed:26091039; MOD_RES 256; /note=S-cysteinyl cysteine; MOD_RES 383; /note=Phosphoserine; /evidence=ECO:0007744|PubMed:24275569
Post Translational Modification PTM: N-glycosylated. Differential glycosylation produces a number of isoforms. N-linked glycan at Asn-107 is alternatively di-antennary, tri-antennary or tetra-antennary. The glycan at Asn-70 is di-antennary with trace amounts of tri-antennary. Glycan at Asn-271 is exclusively di-antennary. Structure of glycans at Asn-70 and Asn-271 is Hex5HexNAc4. The structure of the antennae is Neu5Ac(alpha1-6)Gal(beta1-4)GlcNAc attached to the core structure Man(alpha1-6)[Man(alpha1-3)]Man(beta1-4)GlcNAc(beta1-4)GlcNAc. Some antennae are fucosylated, which forms a Lewis-X determinant. {ECO:0000269|PubMed:12754519, ECO:0000269|PubMed:14760718, ECO:0000269|PubMed:15084671, ECO:0000269|PubMed:16263699, ECO:0000269|PubMed:16335952, ECO:0000269|PubMed:16622833, ECO:0000269|PubMed:19139490, ECO:0000269|PubMed:19159218, ECO:0000269|PubMed:19838169, ECO:0000269|PubMed:22171320, ECO:0000269|PubMed:23826168}.; PTM: Proteolytic processing may yield the truncated form that ranges from Asp-30 to Lys-418.; PTM: (Microbial infection) Proteolytically processed by Staphylococcus aureus seryl, cysteinyl, and metallo-proteases. {ECO:0000269|PubMed:3533918}.
Signal Peptide SIGNAL 1..24; /evidence=ECO:0000269|PubMed:1906855
Structure 3D X-ray crystallography (36)
Cross Reference PDB 1ATU; 1D5S; 1EZX; 1HP7; 1IZ2; 1KCT; 1OO8; 1OPH; 1PSI; 1QLP; 1QMB; 2D26; 2QUG; 3CWL; 3CWM; 3DRM; 3DRU; 3NDD; 3NDF; 3NE4; 3T1P; 4PYW; 5IO1; 5NBU; 5NBV; 6HX4; 6I4V; 6I7U; 6IAY; 6ROD; 7AEL; 7API; 7NPK; 7NPL; 8API; 9API;
Mapped Pubmed ID 10543942; 10559958; 10652252; 10679020; 10747087; 10825291; 10903204; 10915743; 10982397; 11034936; 11035026; 11131449; 11148177; 11199103; 11285137; 11295654; 11306556; 11436564; 11562937; 11744816; 11754061; 11773044; 11785295; 11834734; 11848724; 11862702; 11927603; 11936950; 11991955; 12009885; 12023831; 12023832; 12083479; 12223217; 12235121; 12324297; 12368052; 12372062; 12452840; 12460583; 12464659; 12464660; 12492914; 12498804; 12514663; 12528263; 12578879; 12608432; 12649292; 12689922; 12786756; 12815101; 12815594; 12878320; 12879958; 12893950; 12933574; 12934194; 1349285; 14523999; 14551891; 14616761; 14639110; 14766206; 14766207; 14968215; 15079023; 15100318; 15266208; 15271689; 15271889; 15454649; 15474273; 15498560; 15532029; 15612581; 15653097; 15659365; 15681822; 15709777; 15775753; 15820772; 15820782; 15853774; 15927063; 15994391; 16011217; 16029614; 16044402; 16099106; 16129679; 16137891; 16172282; 16175011; 16179568; 16183649; 16204591; 16278826; 16303754; 16312203; 16321984; 16339299; 16385367; 16387939; 16501672; 16531799; 16540089; 16581345; 16617455; 16624868; 16759229; 16802007; 16819398; 16859601; 16875754; 16887359; 16900211; 16954950; 16979136; 17003923; 17005010; 17006922; 17038314; 17192411; 17203708; 17204961; 17237579; 17240615; 17261591; 17287728; 17316621; 17380188; 17573378; 17635928; 17654345; 17659342; 17660256; 17688648; 17727818; 17855650; 17906067; 17972336; 18025080; 18028276; 18031952; 18060368; 18089349; 18164971; 18258845; 18264947; 18283111; 18294358; 18309698; 18340647; 18353624; 18433707; 18495745; 18504338; 18515255; 18519826; 18534053; 18550704; 18609127; 18624398; 18686007; 18723439; 18725338; 18766166; 18780818; 18843296; 18931508; 19010440; 19073710; 19115690; 19129321; 19164740; 19180336; 19181511; 19197072; 19232354; 19251783; 19251947; 19292870; 19306859; 19344972; 19368725; 19398551; 19417089; 19437508; 19515129; 19596235; 19628210; 19638414; 19656848; 19657220; 19729499; 19738092; 19747908; 19751191; 19763368; 19789190; 19794310; 19815548; 19853488; 19902353; 19913121; 19944704; 19948975; 19956452; 19961268; 20049513; 20086110; 20108056; 20109307; 20119870; 20150872; 20170533; 20217867; 20226649; 20228200; 20298391; 20360068; 20371432; 20379614; 20425789; 20428906; 20435667; 20463814; 20477988; 20521180; 20533886; 20545907; 20558996; 20561599; 20595457; 20628086; 20634282; 20666480; 20667823; 20679433; 20739079; 20811639; 20827781; 20886426; 20953506; 20966969; 21060150; 21067581; 21134340; 21138571; 21173712; 21258324; 21263074; 21273701; 21335450; 21470421; 21477074; 21478858; 21505264; 21532587; 21575917; 21670848; 21698349; 21702661; 21727732; 21752289; 21763297; 21786332; 21791445; 21821620; 21832065; 21843112; 21909074; 21925577; 21976666; 21988832; 22013193; 22075288; 22115549; 22139150; 22157747; 22162908; 22184992; 22215832; 22274560; 22356581; 22363634; 22392975; 22414631; 22544422; 22555971; 22590577; 22634621; 22675024; 22708402; 22723858; 22735536; 22746289; 22793939; 22821488; 22841602; 22896658; 22912729; 22916037; 22933512; 22971141; 22993338; 22995909; 23017899; 23067211; 23088986; 23144969; 23154425; 23226468; 23251618; 23258471; 23299157; 23300094; 23342621; 23376655; 23378591; 23381957; 23479643; 23604680; 23640497; 23648095; 23650620; 23698585; 23717456; 23744167; 23766346; 23829472; 23837941; 23953881; 24011266; 24047901; 24097797; 24119662; 24122823; 24140600; 24213563; 24286083; 24428606; 24449466; 24502947; 24518491; 24582784; 24659849; 24713750; 24743137; 24760148; 24825900; 24840902; 24848503; 24886427; 24892502; 24917561; 25010111; 25070245; 25105300; 25181470; 25211665; 25216870; 25218041; 25242242; 25287719; 25329061; 25351931; 25389308; 25406594; 25425243; 25451265; 25454901; 25487697; 25586378; 25619252; 25658455; 25659085; 25660456; 25667209; 2567291; 25677901; 25738741; 25961766; 25969357; 26003074; 26005342; 26011795; 26091018; 26141700; 26158350; 26174136; 26206901; 26221769; 26243289; 26295339; 26310624; 26363050; 26371243; 26444279; 26634430; 26647313; 26772976; 26831755; 26852026; 26945157; 26947874; 26987331; 27034286; 27102560; 27184740; 27245439; 27246852; 27296815; 27492143; 27515817; 27599294; 27855621; 27877030; 27882547; 27995800; 28004973; 28029757; 28107454; 28121484; 28138235; 28235038; 28243076; 28265093; 28291659; 28362108; 28368395; 28387812; 28409899; 28419579; 28425234; 28440399; 28618946; 28648594; 28652721; 28699171; 28716864; 28825379; 28887821; 28890438; 28922398; 28947017; 29030134; 29049242; 29182883; 29309973; 29324588; 29351445; 29413345; 29460271; 29498931; 29538751; 29573137; 29597895; 29641323; 29679879; 29804144; 29882371; 2989709; 29922281; 29992839; 30134983; 30254761; 30337619; 30351692; 30382774; 30633749; 30739910; 30796179; 30822244; 30833329; 31001247; 31121167; 31183614; 31251477; 31253657; 31281523; 31293581; 31298815; 31307431; 31338581; 31371266; 31450843; 31517326; 31523846; 31661293; 31754242; 31782085; 31892136; 32004242; 32012925; 32019243; 32089881; 32165400; 32232956; 32376409; 32387440; 32417149; 32427833; 32547005; 32699098; 32699193; 32723872; 32725952; 32768500; 32958677; 32960480; 32978262; 33058391; 33192056; 33311470; 33329539; 33512066; 33542414; 33550308; 33556558; 33668611; 33692375; 33753261; 33757485; 33785603; 33858475; 33916165; 33946490; 34073489; 34131090; 34192300; 34199928; 34446826; 34502397; 34638908; 34784346; 34830348; 3486552; 6155027; 6457647; 8467233; 8663406; 9094723; 9150144; 9307973; 9377805; 9395526; 9647643;
Motif
Gene Encoded By
Mass 46,737
Kinetics
Metal Binding
Rhea ID
Cross Reference Brenda