KNG1
entrez
3827
canonical
uniprot
P01042
canonical
ensembl
ENSG00000113889
resolver
ensp
ENSP00000287611
resolver
ensp
ENSP00000396025
resolver
ensp
ENSP00000493985
resolver
genesymbol
KNG1
resolver
genesymbol_syn
BDK
resolver
genesymbol_syn
KNG
resolver
hgnc
HGNC:6383
resolver
uniprot
A8K474
resolver
uniprot
B2RCR2
resolver
uniprot
C9JEX1
resolver
uniprot
P01043
resolver
uniprot
Q53EQ0
resolver
uniprot
Q6PAU9
resolver
uniprot
Q7M4P1
resolver
uniprot_entry
KNG1_HUMAN
resolver
uniprot_sec
A8K474
resolver
uniprot_sec
B2RCR2
resolver
uniprot_sec
C9JEX1
resolver
uniprot_sec
P01043
resolver
uniprot_sec
Q53EQ0
resolver
uniprot_sec
Q6PAU9
resolver
uniprot_sec
Q7M4P1
resolver
amino_acid_sequence
MKLITILFLCSRLLLSLTQESQSEEIDCNDKDLFKAVDAALKKYNSQNQSNNQFVLYRITEATKTVGSDTFYSFKYEIKEGDCPVQSGKTWQDCEYKDAAKAATGECTATVGKRSSTKFSVATQTCQITPAEGPVVTAQYDCLGCVHPISTQSPDLEPILRHGIQYFNNNTQHSSLFMLNEVKRAQRQVVAGLNFRITYSIVQTNCSKENFLFLTPDCKSLWNGDTGECTDNAYIDIQLRIASFSQNCDIYPGKDFVQPPTKICVGCPRDIPTNSPELEETLTHTITKLNAENNATFYFKIDNVKKARVQVVAGKKYFIDFVARETTCSKESNEELTESCETKKLGQSLDCNAEVYVVPWEKKIYPTVNCQPLGMISLMKRPPGFSPFRSSRIGEIKEETTVSPPHTSMAPAQDEERDSGKEQGHTRRHDWGHEKQRKHNLGHGHKHERDQGHGHQRGHGLGHGHEQQHGLGHGHKFKLDDDLEHQGGHVLDHGHKHKHGHGHGKHKNKGKKNGKHNGWKTEHLASSSEDSTTPSAQTQEKTEGPTPIPSLAKPGVTVTFSDFQDSDLIATMMPPISPAPIQSDDDWIPDIQIDPNGLSFNPISDFPDTTSPKCPGRPWKSVSEINPTTQMKESYYFDLTDGLS
uniprot
proteins
disease_involvement
DISEASE: 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 variants affecting the gene represented in this entry.; DISEASE: Angioedema, hereditary, 6 (HAE6) [MIM:619363]: A form of angioedema, a disorder characterized by episodic local swelling involving subcutaneous or submucous tissue of the upper respiratory and gastrointestinal tracts, face, extremities, and genitalia. HAE6 is an autosomal dominant form with onset in adulthood. {ECO:0000269|PubMed:31087670, ECO:0000269|PubMed:33114181}. Note=The disease is caused by variants affecting the gene represented in this entry.
uniprot
proteins
function
FUNCTION: Kininogens are inhibitors of thiol proteases. HMW-kininogen plays an important role in blood coagulation by helping to position optimally prekallikrein and factor XI next to factor XII; HMW-kininogen inhibits the thrombin- and plasmin-induced aggregation of thrombocytes. LMW-kininogen inhibits the aggregation of thrombocytes. LMW-kininogen is in contrast to HMW-kininogen not involved in blood clotting.; FUNCTION: [Bradykinin]: The active peptide bradykinin is a potent vasodilatator that is released from HMW-kininogen shows a variety of physiological effects: (A) influence in smooth muscle contraction, (B) induction of hypotension, (C) natriuresis and diuresis, (D) decrease in blood glucose level, (E) it is a mediator of inflammation and causes (E1) increase in vascular permeability, (E2) stimulation of nociceptors (4E3) release of other mediators of inflammation (e.g. prostaglandins), (F) it has a cardioprotective effect (directly via bradykinin action, indirectly via endothelium-derived relaxing factor action). {ECO:0000305|PubMed:4322742, ECO:0000305|PubMed:6055465}.
uniprot
proteins
mass_dalton
71957
uniprot
proteins
post_translational_modification
PTM: [Bradykinin]: Bradykinin is inactivated by ACE, which removes the dipeptide Arg-Phe from its C-terminus. {ECO:0000269|PubMed:4322742, ECO:0000269|PubMed:6055465}.; PTM: Bradykinin is released from kininogen by plasma kallikrein. {ECO:0000305|PubMed:3366244}.; PTM: Hydroxylation of Pro-383 occurs prior to the release of bradykinin. {ECO:0000269|PubMed:3182782, ECO:0000269|PubMed:3366244}.; PTM: Phosphorylated by FAM20C in the extracellular medium. {ECO:0000269|PubMed:26091039}.; PTM: N- and O-glycosylated. O-glycosylated with core 1 or possibly core 8 glycans. {ECO:0000269|PubMed:12754519, ECO:0000269|PubMed:14760718, ECO:0000269|PubMed:16335952, ECO:0000269|PubMed:19139490, ECO:0000269|PubMed:19159218, ECO:0000269|PubMed:19838169, ECO:0000269|PubMed:3484703}.; PTM: (Microbial infection) Bradykinin is generated upon proteolytic cleavage by S.pyogenes SpeB to produce hypotension during septic shock. {ECO:0000269|PubMed:8760820}.
uniprot
proteins
pubmed
12754519
uniprot
proteins
pubmed
14702039
uniprot
proteins
pubmed
14760718
uniprot
proteins
pubmed
15489334
uniprot
proteins
pubmed
15711755
uniprot
proteins
pubmed
16169873
uniprot
proteins
pubmed
16335952
uniprot
proteins
pubmed
16641997
uniprot
proteins
pubmed
17645545
uniprot
proteins
pubmed
19139490
uniprot
proteins
pubmed
19159218
uniprot
proteins
pubmed
19824718
uniprot
proteins
pubmed
19838169
uniprot
proteins
pubmed
2076202
uniprot
proteins
pubmed
24275569
uniprot
proteins
pubmed
26091039
uniprot
proteins
pubmed
2989293
uniprot
proteins
pubmed
2989294
uniprot
proteins
pubmed
31087670
uniprot
proteins
pubmed
3182782
uniprot
proteins
pubmed
33114181
uniprot
proteins
pubmed
3366244
uniprot
proteins
pubmed
3484703
uniprot
proteins
pubmed
3828072
uniprot
proteins
pubmed
4054110
uniprot
proteins
pubmed
4322742
uniprot
proteins
pubmed
4952632
uniprot
proteins
pubmed
6055465
uniprot
proteins
pubmed
6441591
uniprot
proteins
pubmed
7589467
uniprot
proteins
pubmed
8760820
uniprot
proteins
sequence_length
644
uniprot
proteins
subcellular_location
SUBCELLULAR LOCATION: Secreted, extracellular space.
uniprot
proteins