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    KNG1 kininogen 1 [ Homo sapiens ]

    Gene ID: 3827, updated on 11-May-2012

    Summary

    Official Symbol
    KNG1provided by HGNC
    Official Full Name
    kininogen 1provided by HGNC
    Primary source
    HGNC:6383
    See related
    Ensembl:ENSG00000113889; HPRD:01970; MIM:612358; Vega:OTTHUMG00000150348
    Gene type
    protein coding
    RefSeq status
    REVIEWED
    Organism
    Homo sapiens
    Lineage
    Eukaryota; Metazoa; Chordata; Craniata; Vertebrata; Euteleostomi; Mammalia; Eutheria; Euarchontoglires; Primates; Haplorrhini; Catarrhini; Hominidae; Homo
    Also known as
    BDK; KNG
    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]

    Genomic context

    Location :
    3q27
    Sequence :
    Chromosome: 3; NC_000003.11 (186435098..186462199)
    See KNG1 in Epigenomics, MapViewer

    Chromosome 3 - NC_000003.11Genomic Context describing neighboring genes Neighboring gene histidine-rich glycoprotein Neighboring gene histidine-rich glycoprotein pseudogene Neighboring gene proteasome 26S subunit, non-ATPase, 10 pseudogene 2 Neighboring gene G protein pathway suppressor 2 pseudogene

    Genomic regions, transcripts, and products

    Bibliography

    GeneRIFs: Gene References Into Functions What's a GeneRIF?

    Interactions

    Products Interactant Other Gene Complex Source Pubs Description
    P01042 P01019 AGT    HPRD  PubMed  
    P01042 P05067 APP    HPRD  PubMed  
    P01042 P07384 CAPN1    HPRD  PubMed  
    P01042 P17655 CAPN2    HPRD  PubMed  
    P01042 Q9NPY3 CD93    HPRD  PubMed  
    P01042 P15169 CPN1    HPRD  PubMed  
    P01042 P08311 CTSG    HPRD  PubMed  
    P01042 P43235 CTSK    HPRD  PubMed  
    P01042 P07711 CTSL1    HPRD  PubMed  
    P01042 P25774 CTSS    HPRD  PubMed  
    P01042 P08246 ELANE    HPRD  PubMed  
    P01042 P03951 F11    HPRD  PubMed  
    P01042 P00734 F2    HPRD  PubMed  
    P01042 P02792 FTL    HPRD  PubMed  
    P01042 P07359 GP1BA    HPRD  PubMed  
    P01042 Q14520 HABP2    HPRD  PubMed  
    P01042 P05107 ITGB2    HPRD  PubMed  
    P01042 P06870 KLK1    HPRD  PubMed  
    P01042 P03952 KLKB1    HPRD  PubMed  
    P01042 P01042 KNG1    HPRD  PubMed  
    P01042 P04264 KRT1    HPRD  PubMed  
    P01042 Q16819 MEP1A    HPRD  PubMed  
    P01042 Q03405 PLAUR    HPRD  PubMed  
    P01042 P00747 PLG    HPRD  PubMed  
    P01042 P48147 PREP    HPRD  PubMed  
    P01042 Q8TDU9 RXFP4    HPRD  PubMed  
    P01042 P07996 THBS1    HPRD  PubMed  
    P01042 P04004 VTN    HPRD  PubMed  
    BioGRID:110026 BioGRID:116580 CD93    BioGRID  PubMed Reconstituted Complex 
    BioGRID:110026 BioGRID:108306 ELANE    BioGRID  PubMed Biochemical Activity 
    BioGRID:110026 BioGRID:108458 F11    BioGRID  PubMed Reconstituted Complex 
    BioGRID:110026 BioGRID:108789 FTL    BioGRID  PubMed Reconstituted Complex 
    BioGRID:110026 BioGRID:109895 ITGB2    BioGRID  PubMed Reconstituted Complex 
    BioGRID:110026 BioGRID:110018 KLKB1    BioGRID  PubMed Reconstituted Complex 
    BioGRID:110026 BioGRID:110046 KRT1    BioGRID  PubMed Reconstituted Complex 
    BioGRID:110026 BioGRID:111345 PLAUR    BioGRID  PubMed Reconstituted Complex 
    BioGRID:110026 BioGRID:111356 PLG    BioGRID  PubMed Reconstituted Complex 
    BioGRID:110026 BioGRID:113287 VTN    BioGRID  PubMed Reconstituted Complex 

    General gene information

    Markers

    Homology

    Pathways from BioSystems

    • ACE Inhibitor Pathway, organism-specific biosystem (from WikiPathways)
      ACE Inhibitor Pathway, organism-specific biosystemThe core of this pathway was elucidated over a century ago and involves the conversion of angiotensinogen to angiotensin I (Ang I) by renin, its subsequent conversion to angiotensin II (Ang II) by an...
    • Class A/1 (Rhodopsin-like receptors), organism-specific biosystem (from REACTOME)
      Class A/1 (Rhodopsin-like receptors), organism-specific biosystemRhodopsin-like receptors (class A/1) are the largest group of GPCRs and are the best studied group from a functional and structural point of view. They show great diversity at the sequence level and ...
    • Complement and Coagulation Cascades, organism-specific biosystem (from WikiPathways)
      Complement and Coagulation Cascades, organism-specific biosystemBlood coagulation is a series of coordinated and calcium-dependent proenzyme-to-serine protease conversions likely to be localized on the surfaces of activated cells in vivo. It culminates in the for...
    • Complement and coagulation cascades, organism-specific biosystem (from KEGG)
      Complement and coagulation cascades, organism-specific biosystemThe complement system is a proteolytic cascade in blood plasma and a mediator of innate immunity, a nonspecific defense mechanism against pathogens. There are three pathways of complement activation:...
    • Complement and coagulation cascades, conserved biosystem (from KEGG)
      Complement and coagulation cascades, conserved biosystemThe complement system is a proteolytic cascade in blood plasma and a mediator of innate immunity, a nonspecific defense mechanism against pathogens. There are three pathways of complement activation:...
    • Formation of Fibrin Clot (Clotting Cascade), organism-specific biosystem (from REACTOME)
      Formation of Fibrin Clot (Clotting Cascade), organism-specific biosystemThe formation of a fibrin clot at the site of an injury to the wall of a normal blood vessel is an essential part of the process to stop blood loss after vascular injury. The reactions that lead to ...
    • G alpha (i) signalling events, organism-specific biosystem (from REACTOME)
      G alpha (i) signalling events, organism-specific biosystemThe classical signalling mechanism for G alpha (i) is inhibition of the cAMP dependent pathway through inhibition of adenylate cyclase. Decreased production of cAMP from ATP results in decreased act...
    • G alpha (q) signalling events, organism-specific biosystem (from REACTOME)
      G alpha (q) signalling events, organism-specific biosystemThe classic signalling route for G alpha (q) is activation of phospholipase C beta thereby triggering phosphoinositide hydrolysis, calcium mobilization and protein kinase C activation. This provides ...
    • GPCR downstream signaling, organism-specific biosystem (from REACTOME)
      GPCR downstream signaling, organism-specific biosystemG protein-coupled receptors (GPCRs) are classically defined as the receptor, G-protein and downstream effectors, the alpha subunit of the G-protein being the primary signaling molecule. However, it h...
    • GPCR ligand binding, organism-specific biosystem (from REACTOME)
      GPCR ligand binding, organism-specific biosystemThere are more than 800 G-protein coupled receptor (GPCRs) in the human genome, making it the largest receptor superfamily. GPCRs are also the largest class of drug targets, involved in virtually all...
    • Hemostasis, organism-specific biosystem (from REACTOME)
      Hemostasis, organism-specific biosystemHemostasis is a physiological response that culminates in the arrest of bleeding from an injured vessel. Under normal conditions the vascular endothelium supports vasodilation, inhibits platelet adhe...
    • Intrinsic Pathway, organism-specific biosystem (from REACTOME)
      Intrinsic Pathway, organism-specific biosystemThe intrinsic pathway of blood clotting connects interactions among kininogen (high molecular weight kininogen, HK), prekallikrein (PK), and factor XII to the activation of clotting factor X by a ser...
    • Peptide ligand-binding receptors, organism-specific biosystem (from REACTOME)
      Peptide ligand-binding receptors, organism-specific biosystemThese receptors, a subset of the Class A/1 (Rhodopsin-like) family, all bind peptide ligands which include the chemokines, opioids and somatostatins.
    • Platelet activation, signaling and aggregation, organism-specific biosystem (from REACTOME)
      Platelet activation, signaling and aggregation, organism-specific biosystemPlatelet activation begins with the initial binding of adhesive ligands and of the excitatory platelet agonists (released or generated at the sites of vascular trauma) to cognate receptors on the pla...
    • Platelet degranulation, organism-specific biosystem (from REACTOME)
      Platelet degranulation, organism-specific biosystemPlatelets function as exocytotic cells, secreting a plethora of effector molecules at sites of vascular injury. Platelets contain a number of distinguishable storage granules including alpha granules...
    • Response to elevated platelet cytosolic Ca2+, organism-specific biosystem (from REACTOME)
      Response to elevated platelet cytosolic Ca2+, organism-specific biosystemActivation of phospholipase C enzymes results in the generation of second messengers of the phosphatidylinositol pathway. The events resulting from this pathway are a rise in intracellular calcium an...
    • Signal Transduction, organism-specific biosystem (from REACTOME)
      Signal Transduction, organism-specific biosystemSignal transduction is a process in which extracellular signals elicit changes in cell state and activity. Transmembrane receptors sense changes in the cellular environment by binding ligands, such a...
    • Signaling by GPCR, organism-specific biosystem (from REACTOME)
      Signaling by GPCR, organism-specific biosystemG protein-coupled receptors (GPCRs; 7TM receptors; seven transmembrane domain receptors; heptahelical receptors; G protein-linked receptors [GPLR]) are the largest family of transmembrane receptors i...
    • Syndecan-2-mediated signaling events, organism-specific biosystem (from Pathway Interaction Database)
      Syndecan-2-mediated signaling events, organism-specific biosystem
      Syndecan-2-mediated signaling events
    • amb2 Integrin signaling, organism-specific biosystem (from Pathway Interaction Database)
      amb2 Integrin signaling, organism-specific biosystem
      amb2 Integrin signaling

    Gene Ontology Provided by GOA

    Function Evidence Code Pubs
    cysteine-type endopeptidase inhibitor activity IEA
    Inferred from Electronic Annotation
    more info
     
    heparin binding NAS
    Non-traceable Author Statement
    more info
    PubMed 
    peptidase inhibitor activity IEA
    Inferred from Electronic Annotation
    more info
     
    receptor binding IPI
    Inferred from Physical Interaction
    more info
    PubMed 
    zinc ion binding NAS
    Non-traceable Author Statement
    more info
    PubMed 
    Process Evidence Code Pubs
    blood coagulation TAS
    Traceable Author Statement
    more info
     
    blood coagulation, intrinsic pathway TAS
    Traceable Author Statement
    more info
     
    elevation of cytosolic calcium ion concentration IDA
    Inferred from Direct Assay
    more info
    PubMed 
    inflammatory response TAS
    Traceable Author Statement
    more info
    PubMed 
    negative regulation of blood coagulation IDA
    Inferred from Direct Assay
    more info
    PubMed 
    negative regulation of cell adhesion IDA
    Inferred from Direct Assay
    more info
    PubMed 
    platelet activation TAS
    Traceable Author Statement
    more info
     
    platelet degranulation TAS
    Traceable Author Statement
    more info
     
    positive regulation of apoptotic process NAS
    Non-traceable Author Statement
    more info
    PubMed 
    positive regulation of renal sodium excretion TAS
    Traceable Author Statement
    more info
    PubMed 
    positive regulation of urine volume TAS
    Traceable Author Statement
    more info
    PubMed 
    smooth muscle contraction TAS
    Traceable Author Statement
    more info
    PubMed 
    vasodilation IEA
    Inferred from Electronic Annotation
    more info
     
    Component Evidence Code Pubs
    extracellular region NAS
    Non-traceable Author Statement
    more info
    PubMed 
    extracellular region TAS
    Traceable Author Statement
    more info
     
    extracellular space IEA
    Inferred from Electronic Annotation
    more info
     
    plasma membrane TAS
    Traceable Author Statement
    more info
     
    platelet alpha granule lumen TAS
    Traceable Author Statement
    more info
     

    General protein information

    Preferred Names
    kininogen-1
    Names
    kininogen-1
    HMWK
    bradykinin
    fitzgerald factor
    high molecular weight kininogen
    alpha-2-thiol proteinase inhibitor
    williams-Fitzgerald-Flaujeac factor

    NCBI Reference Sequences (RefSeq)

    RefSeqs maintained independently of Annotated Genomes

    These reference sequences exist independently of genome builds. Explain

    These reference sequences are curated independently of the genome annotation cycle, so their versions may not match the RefSeq versions in the current genome build. Identify version mismatches by comparing the version of the RefSeq in this section to the one reported in Genomic regions, transcripts, and products above.

    Genomic

    1. NG_016009.1 RefSeqGene

      Range
      5001..32102
      Download
      GenBank, FASTA, Sequence Viewer (Graphics)

    mRNA and Protein(s)

    1. NM_000893.3NP_000884.1  kininogen-1 isoform 2 precursor

      Status: REVIEWED

      Description
      Transcript Variant: This variant (2) uses an alternate splice pattern in the 3' coding region, compared to variant 1. The resulting isoform (2), also known as low molecular weight preprokininogen, has a shorter and distinct C-terminus, compared to isoform 1.
      Source sequence(s)
      AC109780, AC112907, DC362362, K02566
      Consensus CDS
      CCDS3281.1
      UniProtKB/Swiss-Prot
      P01042
      Related
      ENSP00000287611, OTTHUMP00000197128, ENST00000287611, OTTHUMT00000317737
      Conserved Domains (2) summary
      cd00042
      Location:266370
      Blast Score: 222
      CY; Cystatin-like domain; Cystatins are a family of cysteine protease inhibitors that occur mainly as single domain proteins. However some extracellular proteins such as kininogen, His-rich glycoprotein and fetuin also contain these domains.
      cl09238
      Location:26114
      Blast Score: 164
      CY; Cystatin-like domain; Cystatins are a family of cysteine protease inhibitors that occur mainly as single domain proteins. However some extracellular proteins such as kininogen, His-rich glycoprotein and fetuin also contain these domains.
    2. NM_001102416.2NP_001095886.1  kininogen-1 isoform 1 precursor

      Status: REVIEWED

      Description
      Transcript Variant: This variant (1) represents the longer transcript and encodes the longer isoform (1), also known as high molecular weight preprokininogen.
      Source sequence(s)
      AC109780, AC112907, DC362362, K02566
      Consensus CDS
      CCDS43183.1
      UniProtKB/Swiss-Prot
      P01042
      Related
      ENSP00000265023, OTTHUMP00000197129, ENST00000265023, OTTHUMT00000317738
      Conserved Domains (2) summary
      cd00042
      Location:266370
      Blast Score: 229
      CY; Cystatin-like domain; Cystatins are a family of cysteine protease inhibitors that occur mainly as single domain proteins. However some extracellular proteins such as kininogen, His-rich glycoprotein and fetuin also contain these domains.
      cl09238
      Location:26114
      Blast Score: 169
      CY; Cystatin-like domain; Cystatins are a family of cysteine protease inhibitors that occur mainly as single domain proteins. However some extracellular proteins such as kininogen, His-rich glycoprotein and fetuin also contain these domains.
    3. NM_001166451.1NP_001159923.1  kininogen-1 isoform 3 precursor

      Status: REVIEWED

      Description
      Transcript Variant: This variant (3) uses an alternate splice pattern in the 3' coding region and lacks an alternate in-frame exon, compared to variant 1. The resulting isoform (3) has a shorter and distinct C-terminus and lacks an internal segment, compared to isoform 1.
      Source sequence(s)
      AC112907, CD014130, DC362362
      Consensus CDS
      CCDS54695.1
      UniProtKB/TrEMBL
      C9JEX1
      Related
      ENSP00000396025, OTTHUMP00000210109, ENST00000447445, OTTHUMT00000344651
      Conserved Domains (2) summary
      cd00042
      Location:230334
      Blast Score: 221
      CY; Cystatin-like domain; Cystatins are a family of cysteine protease inhibitors that occur mainly as single domain proteins. However some extracellular proteins such as kininogen, His-rich glycoprotein and fetuin also contain these domains.
      cl09238
      Location:26114
      Blast Score: 164
      CY; Cystatin-like domain; Cystatins are a family of cysteine protease inhibitors that occur mainly as single domain proteins. However some extracellular proteins such as kininogen, His-rich glycoprotein and fetuin also contain these domains.

    RefSeqs of Annotated Genomes: Build 37.3

    The following sections contain reference sequences that belong to a specific genome build. Explain

    Reference GRCh37.p5 Primary Assembly

    Genomic

    1. NC_000003.11 Reference GRCh37.p5 Primary Assembly

      Range
      186435098..186462199
      Download
      GenBank, FASTA, Sequence Viewer (Graphics)

    Alternate HuRef

    Genomic

    1. AC_000135.1 Alternate HuRef

      Range
      183844775..183871713
      Download
      GenBank, FASTA, Sequence Viewer (Graphics)

    Related Sequences

    Nucleotide Protein
    Heading Accession and Version
    genomic AC109780.7 (57757..59093) None
    genomic AC112907.7 (105127..130870) None
    genomic AY248697.1 AAO61092.1
    genomic CH471052.2 EAW78179.1
      EAW78180.1
      EAW78181.1
    genomic HC918468.1 CBN73461.1
    genomic M11437.1 AAB59550.1
      AAB59551.1
    genomic M11438.1 AAB59550.1
    genomic M11521.1 AAB59550.1
    genomic M11522.1 AAB59550.1
    genomic M11523.1 AAB59550.1
    genomic M11524.1 AAB59550.1
    genomic M11525.1 AAB59550.1
    genomic M11526.1 AAB59550.1
    genomic M11527.1 AAB59550.1
    genomic M11528.1 AAB59550.1
    mRNA AI133186.1 None
    mRNA AK223589.1 BAD97309.1
    mRNA AK290839.1 BAF83528.1
    mRNA AK298440.1 BAG60660.1
    mRNA AK303768.1 BAG64734.1
    mRNA AK315230.1 BAG37659.1
    mRNA BC026253.1 AAH26253.1
    mRNA BC060039.1 AAH60039.1
    mRNA BG430928.1 None
    mRNA CB148843.1 None
    mRNA CD014130.1 None
    mRNA DA537547.1 None
    mRNA DC362362.1 None
    mRNA K02566.1 AAA35497.1
    Protein Accession Links
    GenPept Link UniProtKB Link
    P01042.2 GenPept UniProtKB/Swiss-Prot:P01042
    Q05CF8 GenPept UniProtKB/TrEMBL:Q05CF8

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