catalog number :
MBS000059
products full name :
Rabbit KNG1 (heavy chain, Cleaved-Lys380) Antibody
products short name :
KNG1 (heavy chain, Cleaved-Lys380)
other names :
kininogen-1 isoform 2; Kininogen-1; kininogen-1; HMWK; bradykinin; fitzgerald factor; high molecular weight kininogen; alpha-2-thiol proteinase inhibitor; williams-Fitzgerald-Flaujeac factor; kininogen 1; Alpha-2-thiol proteinase inhibitor; Fitzgerald factor; High molecular weight kininogen; HMWK; Williams-Fitzgerald-Flaujeac factorCleaved into the following 6 chains:Kininogen-1 heavy chainT-kinin; Alternative name(s):; Ile-Ser-BradykininBradykinin; Alternative name(s):; Kallidin ILysyl-bradykinin; Alternative name(s):; Kallidin II
products gene name :
KNG1
other gene names :
KNG1; KNG1; BK; BDK; KNG; BDK; KNG; HMWK
uniprot entry name :
KNG1_HUMAN
form :
Phosphate buffered saline (without Mg2+ and Ca2+), pH 7.4, 150mM NaCl, 0.02% sodium azide and 50% glycerol
tested application :
ELISA (EIA), Western Blot (WB)
ncbi acc num :
NP_000884.1
ncbi gb acc num :
NM_000893.3
ncbi mol weight :
71,957 Da
ncbi pathways :
ACE Inhibitor Pathway (198763); Class A/1 (Rhodopsin-like Receptors) Pathway (106357); Complement And Coagulation Cascades Pathway (198880); Complement And Coagulation Cascades Pathway (83073); Complement And Coagulation Cascades Pathway (484); Formation Of Fibrin Clot (Clotting Cascade) Pathway (106057); G Alpha (i) Signalling Events Pathway (119550); G Alpha (q) Signalling Events Pathway (106043); GPCR Downstream Signaling Pathway (119548); GPCR Ligand Binding Pathway (161020)
ncbi summary :
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. 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, Oct 2009]
uniprot summary :
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. Defects in KNG1 are the cause of high molecular weight kininogen deficiency (HMWK deficiency). 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. 2 isoforms of the human protein are produced by alternative splicing. Protein type: Cell adhesion; Secreted; Inhibitor; Secreted, signal peptide; Contractile; Motility/polarity/chemotaxis. Chromosomal Location of Human Ortholog: 3q27. Cellular Component: extracellular space; plasma membrane; extracellular region. Molecular Function: heparin binding; protein binding; zinc ion binding; cysteine protease inhibitor activity; receptor binding. Biological Process: negative regulation of proteolysis; platelet activation; elevation of cytosolic calcium ion concentration; smooth muscle contraction; platelet degranulation; positive regulation of apoptosis; negative regulation of blood coagulation; negative regulation of cell adhesion; blood coagulation; inflammatory response; vasodilation; blood coagulation, intrinsic pathway. Disease: High Molecular Weight Kininogen Deficiency