| Predicate | Object |
|---|---|
| rdf:type | |
| biopax3:comment |
FUNCTION: (1) Kininogens are inhibitors of thiol proteases; (2) HMW-kininogen plays an important role in blood coagulation by helping to position optimally prekallikrein and factor XI next to factor XII; (3) HMW-kininogen inhibits the thrombin- and plasmin- induced aggregation of thrombocytes; (4) the active peptide bradykinin that is released from HMW-kininogen shows a variety of physiological effects: (4A) influence in smooth muscle contraction, (4B) induction of hypotension, (4C) natriuresis and diuresis, (4D) decrease in blood glucose level, (4E) it is a mediator of inflammation and causes (4E1) increase in vascular permeability, (4E2) stimulation of nociceptors (4E3) release of other mediators of inflammation (e.g. prostaglandins), (4F) it has a cardioprotective effect (directly via bradykinin action, indirectly via endothelium-derived relaxing factor action); (5) LMW-kininogen inhibits the aggregation of thrombocytes; (6) LMW- kininogen is in contrast to HMW-kininogen not involved in blood clotting. SUBCELLULAR LOCATION: Secreted, extracellular space. ALTERNATIVE PRODUCTS: Event=Alternative splicing; Named isoforms=2; Name=HMW; IsoId=P01042-1; Sequence=Displayed; Name=LMW; IsoId=P01042-2; Sequence=VSP_001261, VSP_001262; TISSUE SPECIFICITY: Secreted in plasma. T-kinin is detected in malignant ovarian, colon and breast carcinomas, but not in benign tumors. PTM: Bradykinin is released from kininogen by plasma kallikrein. PTM: Hydroxylation of Pro-383 occurs prior to the release of bradykinin. PTM: Phosphorylation sites are present in the extracelllular medium. PTM: N- and O-glycosylated. O-glycosylated with core 1 or possibly core 8 glycans. POLYMORPHISM: The T-kinin peptide is missing residues 378 to 380, probably as a result of a naturally occurring variant. The complete sequence of the T-kinin peptide is therefore ISRPPGFSPFR. This peptide is associated with malignant tumors but not with benign ones. DISEASE: Defects in KNG1 are the cause of high molecular weight kininogen deficiency (HMWK deficiency) [MIM:228960]. HMWK deficiency is an autosomal recessive coagulation defect. Patients with HWMK deficiency do not have a hemorrhagic tendency, but they exhibit abnormal surface-mediated activation of fibrinolysis. SIMILARITY: Contains 3 cystatin domains. WEB RESOURCE: Name=Wikipedia; Note=High molecular weight kininogen entry; URL="http://en.wikipedia.org/wiki/High-molecular_weight_kininogen"; WEB RESOURCE: Name=SeattleSNPs; URL="http://pga.gs.washington.edu/data/kng/"; GENE SYNONYMS:KNG1 BDK KNG. COPYRIGHT: Protein annotation is derived from the UniProt Consortium (http://www.uniprot.org/). Distributed under the Creative Commons Attribution-NoDerivs License.,
SEQUENCE 644 AA; 71957 MW; 3132B4DF2954C24E CRC64;
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| biopax3:xref |
urn:biopax:RelationshipXref:HGNC_HGNC:6383,
urn:biopax:RelationshipXref:NCBI GENE_3827,
urn:biopax:RelationshipXref:REFSEQ_NP_000884,
urn:biopax:RelationshipXref:REFSEQ_NP_001095886,
urn:biopax:UnificationXref:UNIPROT_A8K474,
urn:biopax:UnificationXref:UNIPROT_B2RCR2,
urn:biopax:UnificationXref:UNIPROT_P01042,
urn:biopax:UnificationXref:UNIPROT_P01043,
urn:biopax:UnificationXref:UNIPROT_Q53EQ0,
urn:biopax:UnificationXref:UNIPROT_Q6PAU9,
urn:biopax:UnificationXref:UNIPROT_Q7M4P1
|
| biopax3:displayName |
KNG1_HUMAN
|
| biopax3:name |
Alpha-2-thiol proteinase inhibitor,
Bradykinin,
Fitzgerald factor,
HMWK,
High molecular weight kininogen,
Ile-Ser-Bradykinin,
KNG1,
Kallidin I,
Kallidin II,
Kininogen-1 heavy chain,
Kininogen-1 light chain,
Low molecular weight growth-promoting factor,
Lysyl-bradykinin,
T-kinin,
Williams-Fitzgerald-Flaujeac factor
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| biopax3:entityFeature | |
| biopax3:organism | |
| biopax3:sequence |
MKLITILFLCSRLLLSLTQESQSEEIDCNDKDLFKAVDAALKKYNSQNQSNNQFVLYRITEATKTVGSDTFYSFKYEIKEGDCPVQSGKTWQDCEYKDAAKAATGECTATVGKRSSTKFSVATQTCQITPAEGPVVTAQYDCLGCVHPISTQSPDLEPILRHGIQYFNNNTQHSSLFMLNEVKRAQRQVVAGLNFRITYSIVQTNCSKENFLFLTPDCKSLWNGDTGECTDNAYIDIQLRIASFSQNCDIYPGKDFVQPPTKICVGCPRDIPTNSPELEETLTHTITKLNAENNATFYFKIDNVKKARVQVVAGKKYFIDFVARETTCSKESNEELTESCETKKLGQSLDCNAEVYVVPWEKKIYPTVNCQPLGMISLMKRPPGFSPFRSSRIGEIKEETTVSPPHTSMAPAQDEERDSGKEQGHTRRHDWGHEKQRKHNLGHGHKHERDQGHGHQRGHGLGHGHEQQHGLGHGHKFKLDDDLEHQGGHVLDHGHKHKHGHGHGKHKNKGKKNGKHNGWKTEHLASSSEDSTTPSAQTQEKTEGPTPIPSLAKPGVTVTFSDFQDSDLIATMMPPISPAPIQSDDDWIPDIQIDPNGLSFNPISDFPDTTSPKCPGRPWKSVSEINPTTQMKESYYFDLTDGLS
|
| biopax3:standardName |
Kininogen-1
|