-
别名
- Alpha-2-thiol proteinase inhibitor
- BDK
- BK
- Fitzgerald factor
- High molecular weight kininogen
- HMWK
- Ile-Ser-Bradykinin
- Kallidin I
- Kallidin II
- Kininogen 1
- KNG
- KNG1
- KNG1_HUMAN
- LMW
- Low molecular weight growth-promoting factor
- Low molecular weight kininogen
- Williams-Fitzgerald-Flaujeac factor
see all
-
功能
(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.
-
组织特异性
Secreted in plasma. T-kinin is detected in malignant ovarian, colon and breast carcinomas, but not in benign tumors.
-
疾病相关
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.
-
序列相似性
Contains 3 cystatin kininogen-type domains.
-
翻译后修饰
Bradykinin is released from kininogen by plasma kallikrein.
Hydroxylation of Pro-383 occurs prior to the release of bradykinin.
Phosphorylation sites are present in the extracellular medium.
N- and O-glycosylated. O-glycosylated with core 1 or possibly core 8 glycans.
-
细胞定位
Secreted > extracellular space.
-
Information by UniProt