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
|
|
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 |
|
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
|
|
|
|
Molecular Function |
|
Biological Process |
|
Cellular Component |
|
PDB ID |
|
InterPro |
IPR000010
Proteinase inhibitor I25, cystatin
IPR002395
HMW kininogen
|
PFAM |
PF00031
|
PRINTS |
PR00334
|
PIRSF |
|
SMART |
SM00043
|
TIGRFAMs |
|
Modification |
|
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
|
|
|