KNG1 Gene

Last updated on: 01.09.2022

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The KNG1 gene (KNG1 stands for kininogen 1) is a protein-coding gene located on chromosome 3q27.3. Three transcript variants encoding different isoforms have been found for this gene. The KNG1 gene produces by alternative splicing two different proteins

  • high molecular weight kininogen (HMWK)


  • low molecular weight kininogen (LMWK).

HMWK is important for blood clotting and the assembly of the kallikrein-kinin system. In addition, HMWK releases bradykinin, a peptide with numerous physiological effects. Bradykinin also functions as an antimicrobial peptide with antibacterial and antifungal effects. Unlike HMWK, LMWK is not involved in blood clotting.

General information
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Kininogens are inhibitors of thiol proteases. HMW kininogen plays an important role in blood coagulation by helping to optimally position prekallikrein and factor XI next to factor XII. HMW-kininogen inhibits thrombin- and plasmin-induced aggregation of platelets. The active peptide bradykinin released from HMW kininogen exhibits a variety of physiological effects: such as a cardioprotective effect (directly via the action of bradykinin, indirectly via the action of endothelium-derived relaxing factor; LMW kininogen inhibits platelet aggregation). LMW kininogen, unlike HMW kininogen, is not involved in blood clotting.

Clinical picture
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Diseases associated with KNG1 include:


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Severe acute respiratory syndrome coronavirus 2 (SARS-CoV-2) infection reduces or depletes angiotensin-converting enzyme 2 (ACE2), leading to an increase in des-Arg(9)-bradykinin, a bioactive metabolite of bradykinin associated with lung injury and inflammation.

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  1. Bork K et al. (2019) Hereditary angioedema cosegregating with a novel kininogen 1 gene mutation changing the N-terminal cleavage site of bradykinin. (Letter) Allergy 74: 2479-2544.
  2. Hayashi H et al. (1990) Molecular genetic survey of five Japanese families with high molecular weight kininogen deficiency. Blood 75: 1296-1304.
  3. Krijanovski Y et al. (2003) Characterization of molecular defects of Fitzgerald trait and another novel high-molecular-weight kininogen-deficient patient: insights into structural requirements for kininogen expression. Blood 101: 4430-4436.
  4. Lefrere JJ et al (1986) A new case of high molecular weight kininogen inherited deficiency. Am J Hemat 22: 415-419.
  5. Loules G et al (2020) Deciphering the genetics of primary angioedema with normal levels of C1 inhibitor. J Clin Med 9: 3402.

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Last updated on: 01.09.2022