Kininogen 1

Kininogen-1 (KNG1), also known as alpha-2-thiol proteinase inhibitor, Williams-Fitzgerald-Flaujeac factor or the HMWK-kallikrein factor is a protein^[5] that in humans is encoded by the KNG1 gene.^[6][7] Kininogen-1 is the precursor protein to high-molecular-weight kininogen (HMWK), low-molecular-weight kininogen (LMWK), and bradykinin.^[5]

KNG1
Available structures PDB Human UniProt search: PDBe RCSB List of PDB id codes 1NY2, 2WOK, 4ASQ, 4ASR, 4ECB, 4ECC, 5I25
Identifiers
Aliases	KNG1, BDK, BK, KNG, Kininogen 1, HMWK, HAE6, HK
External IDs	OMIM: 612358; MGI: 3027157; HomoloGene: 88343; GeneCards: KNG1; OMA:KNG1 - orthologs
Gene location (Human) Chr. Chromosome 3 (human)[1] Band 3q27.3 Start 186,717,348 bp[1] End 186,744,410 bp[1]
Gene location (Mouse) Chr. Chromosome 16 (mouse)[2] Band 16|16 B1 Start 22,804,604 bp[2] End 22,848,232 bp[2]
RNA expression pattern Bgee Human Mouse (ortholog) Top expressed in renal medulla liver right lobe of liver kidney tubule metanephric glomerulus human kidney testicle pancreatic ductal cell rectum prefrontal cortex Top expressed in right kidney human kidney renal cortex proximal tubule liver bone marrow embryo morula placenta esophagus More reference expression data BioGPS More reference expression data
Gene ontology Molecular function peptidase inhibitor activity heparin binding zinc ion binding protein binding signaling receptor binding cysteine-type endopeptidase inhibitor activity Cellular component blood microparticle plasma membrane extracellular exosome platelet alpha granule lumen extracellular space endoplasmic reticulum lumen extracellular matrix extracellular region collagen-containing extracellular matrix Biological process hemostasis blood coagulation, intrinsic pathway negative regulation of proteolysis negative regulation of cell adhesion negative regulation of peptidase activity negative regulation of blood coagulation positive regulation of cytosolic calcium ion concentration platelet degranulation vasodilation positive regulation of apoptotic process inflammatory response blood coagulation negative regulation of endopeptidase activity post-translational protein modification G protein-coupled receptor signaling pathway Sources:Amigo / QuickGO
Orthologs Species Human Mouse Entrez 3827 385643 Ensembl ENSG00000113889 ENSMUSG00000060459 UniProt P01042 n/a RefSeq (mRNA) NM_001166451 NM_000893 NM_001102416 NM_001102409 NM_001102410 NM_201375 RefSeq (protein) NP_000884 NP_001095886 NP_001159923 n/a Location (UCSC) Chr 3: 186.72 – 186.74 Mb Chr 16: 22.8 – 22.85 Mb PubMed search [3] [4]
Wikidata
View/Edit Human View/Edit Mouse

Expression

The KNG1 gene uses alternative splicing to generate two different proteins: high-molecular-weight kininogen (HMWK) and low-molecular-weight kininogen (LMWK). HMWK in turn is cleaved by the enzyme kallikrein to produce bradykinin.

• KNG1 gene → low-molecular-weight kininogen (LMWK) protein (contains 427 amino acids) or high-molecular-weight kininogen (HMWK) protein (644 amino acids)
• HMWK protein → bradykinin peptide (9 amino acids)

Function

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.^[6]

Kininogen-1 is a constituent of the blood coagulation system as well as the kinin-kallikrein system.

See also

• high-molecular-weight kininogen
• low-molecular-weight kininogen
• bradykinin