Summary of KNG1
The gene codes for a protein, kininogen 1. It generates HMWK and LMWK. HMWK is used for blood coagulation, but LMWK is not [R].
The Function of KNG1
(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.
Protein names
Recommended name:
Kininogen-1Short name:
HMWKAlternative name(s):
Alpha-2-thiol proteinase inhibitorFitzgerald factor
High molecular weight kininogen
Williams-Fitzgerald-Flaujeac factor
Ile-Ser-Bradykinin
Kallidin I
Kallidin II
- RS11924390 (KNG1) ??
- RS1656966 (KNG1) ??
- RS4686799 (KNG1) ??
- RS5030062 (KNG1) ??
- RS698078 (KNG1) ??
- RS710446 (KNG1) ??
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Top Gene-Substance Interactions
KNG1 Interacts with These Diseases
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Substances That Increase KNG1
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Substances That Decrease KNG1
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Advanced Summary
From NCBI Gene: High molecular weight kininogen deficiencyFrom UniProt: High molecular weight kininogen deficiency (HMWK deficiency): Autosomal recessive coagulation defect. Patients with HWMK deficiency do not have a hemorrhagic tendency, but they exhibit abnormal surface-mediated activation of fibrinolysis. [MIM:228960]
From NCBI Gene: This gene uses alternative splicing to generate two different proteins- high molecular weight kininogen (HMWK) and low molecular weight kininogen (LMWK). HMWK is essential for blood coagulation and assembly of the kallikrein-kinin system. Also, bradykinin, a peptide causing numerous physiological effects, is released from HMWK. Bradykinin also functions as an antimicrobial peptide with antibacterial and antifungal activity. In contrast to HMWK, LMWK is not involved in blood coagulation. Three transcript variants encoding different isoforms have been found for this gene.[provided by RefSeq, Nov 2014] From UniProt: (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.
Conditions with Increased Gene Activity
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Conditions with Decreased Gene Activity
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Technical
The following transcription factors affect gene expression:
Tissue specificity:
Secreted in plasma. T-kinin is detected in malignant ovarian, colon and breast carcinomas, but not in benign tumors.
Gene Pathways:
Molecular Function:
Biological Processes:
- Blood Coagulation, Intrinsic Pathway
- Inflammatory Response
- Negative Regulation Of Blood Coagulation
- Negative Regulation Of Cell Adhesion
- Negative Regulation Of Proteolysis
- Platelet Degranulation
- Positive Regulation Of Apoptotic Process
- Positive Regulation Of Cytosolic Calcium Ion Concentration
- Vasodilation