Homo sapiens Protein: KNG1
Summary
InnateDB Protein IDBP-69063.6
Last Modified 2014-10-13 [Report errors or provide feedback]
Gene Symbol KNG1
Protein Name kininogen 1
Synonyms BDK; BK; KNG;
Species Homo sapiens
Ensembl Protein ENSP00000265023
InnateDB Gene IDBG-69059 (KNG1)
Protein Structure
UniProt Annotation
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 Localization Secreted, extracellular space.
Disease Associations High molecular weight kininogen deficiency (HMWK deficiency) [MIM:228960]: Autosomal recessive coagulation defect. Patients with HWMK deficiency do not have a hemorrhagic tendency, but they exhibit abnormal surface-mediated activation of fibrinolysis. Note=The disease is caused by mutations affecting the gene represented in this entry.
Tissue Specificity Secreted in plasma. T-kinin is detected in malignant ovarian, colon and breast carcinomas, but not in benign tumors. {ECO:0000269PubMed:2076202}.
Comments
Interactions
Number of Interactions This gene and/or its encoded proteins are associated with 22 experimentally validated interaction(s) in this database.
Experimentally validated
Total 22 [view]
Protein-Protein 20 [view]
Protein-DNA 2 [view]
Protein-RNA 0
DNA-DNA 0
RNA-RNA 0
DNA-RNA 0
Gene Ontology

Molecular Function
Accession GO Term
GO:0004869 cysteine-type endopeptidase inhibitor activity
GO:0005102 receptor binding
GO:0005515 protein binding
GO:0008201 heparin binding
GO:0008270 zinc ion binding
Biological Process
GO:0002576 platelet degranulation
GO:0006939 smooth muscle contraction
GO:0006954 inflammatory response
GO:0007162 negative regulation of cell adhesion
GO:0007204 positive regulation of cytosolic calcium ion concentration
GO:0007596 blood coagulation
GO:0007597 blood coagulation, intrinsic pathway
GO:0010951 negative regulation of endopeptidase activity
GO:0030168 platelet activation
GO:0030195 negative regulation of blood coagulation
GO:0035810 positive regulation of urine volume
GO:0035815 positive regulation of renal sodium excretion
GO:0042311 vasodilation
GO:0043065 positive regulation of apoptotic process
GO:0045861 negative regulation of proteolysis
Cellular Component
GO:0005576 extracellular region
GO:0005615 extracellular space
GO:0005886 plasma membrane
GO:0031093 platelet alpha granule lumen
GO:0070062 extracellular vesicular exosome
GO:0072562 blood microparticle
Protein Structure and Domains
PDB ID
InterPro IPR000010 Proteinase inhibitor I25, cystatin
IPR002395 HMW kininogen
PFAM PF00031
PRINTS PR00334
PIRSF
SMART SM00043
TIGRFAMs
Post-translational Modifications
Modification
Cross-References
SwissProt P01042
PhosphoSite PhosphoSite-P01042
TrEMBL
UniProt Splice Variant
Entrez Gene 3827
UniGene Hs.77741
RefSeq NP_001095886
HUGO HGNC:6383
OMIM 612358
CCDS CCDS43183
HPRD 01970
IMGT
EMBL AC109780 AC112907 AK223589 AK290839 AK315230 AY248697 BC060039 CH471052 K02566 M11437 M11438 M11521 M11522 M11523 M11524 M11525 M11526 M11527 M11528
GenPept AAA35497 AAB59550 AAB59551 AAH60039 AAO61092 BAD97309 BAF83528 BAG37659 EAW78179