プレカリクレイン
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出典(authority):フリー百科事典『ウィキペディア(Wikipedia)』「2013/11/19 23:25:02」(JST)
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Prekallikrein (PK), also known as Fletcher factor, is an 85,000 Mr serine protease that complexes with high-molecular-weight kininogen. PK is the precursor of plasma kallikrein, which is a serine protease that activates kinins. PK is cleaved to produce kallikrein by activated Factor XII (Hageman factor). [1]
Prekallikrein deficiency[edit]
Hereditary deficiencies in PK are very rare. They can cause a prolonged APTT, which can be corrected by incubation of the patient’s plasma.
Deficiencies in PK can also be acquired due to some disease states, such as angioedema, infection, DIC, and sickle-cell disease.[1]
Although most cases of prekallikrein deficiency are asymptomatic, a few reports link severe prekallikrein deficiency with thrombotic phenomena and recurrent pregnancy loss. More recently, a case of prekallikrein deficiency was shown to be associated with severe mucosal bleeding.[2]
Discovery of prekallikrein[edit]
PK was initially described by Hathaway et al. in 1965 after encountering a Kentucky family who exhibited strikingly abnormal APTT results, but showed no bleeding symptoms. The family appeared to have a hereditary deficiency in an unknown coagulation factor, dubbed “Fletcher factor” after the family. In 1973 Kirk Wuepper determined that Fletcher factor and prekallikrein were the same.[3]
References[edit]
- ^ a b Goodnight, SH Jr, Hathaway, WE. Disorders of Hemostasis and Thrombosis. Second edition. 2001.
- ^ Dasanu CA, et al. "A Case of Prekallikrein Deficiency Resulting in Severe Recurrent Mucosal Hemorrhage". American Journal of Medical Sciences, 2009.
- ^ OMIM article on prekallikrein deficiency
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Proteins: coagulation
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| Coagulation factors |
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Primary hemostasis
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- platelet membrane glycoproteins: Ib (A
- B
- IX)
- IIb/IIIa (IIb
- IIIa)
- VI
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Intrinsic pathway
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- HMWK/Bradykinin
- Prekallikrein/Kallikrein
- XII "Hageman"
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Extrinsic pathway
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Common pathway
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- X
- V
- II "(Pro)thrombin"
- I "Fibrin"
- Fibrinogen (FGA, FGG)
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| Coagulation inhibitors |
- Antithrombin (inhibits II, IX, X, XI, XII)
- Protein C (inhibits V, VIII)/Protein S (cofactor for protein C)
- Protein Z (inhibits X)
- ZPI (inhibits X, XI)
- TFPI (inhibits III)
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| Thrombolysis/fibrinolysis |
- Plasmin
- tPA/urokinase
- PAI-1/2
- α2-AP
- α2-macroglobulin
- TAFI
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cell/phys (coag, heme, immu, gran), csfs
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rbmg/mogr/tumr/hist, sysi/epon, btst
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drug (B1/2/3+5+6), btst, trns
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UpToDate Contents
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English Journal
- PF-04886847 (an Inhibitor of Plasma Kallikrein) Attenuates Inflammatory Mediators and Activation of Blood Coagulation in Rat Model of Lipopolysaccharide (LPS) - Induced Sepsis.
- Kolte D, Bryant JW, Gibson GW, Wang J, Shariat-Madar Z.SourceThe University of Mississippi, University, MS 38677-1848, USA. madar@olemiss.edu.
- Cardiovascular & hematological agents in medicinal chemistry.Cardiovasc Hematol Agents Med Chem.2012 Jun 1;10(2):154-66.
- The plasma kallikrein-mediated proteolysis regulates both thrombosis and inflammation. Previous study has shown that PF-04886847 is a potent and competitive inhibitor of kallikrein, suggesting that it might be useful for the treatment of kallikrein-kinin mediated inflammatory and thrombotic disorder
- PMID 22352684
- Plasma Prolylcarboxypeptidase (Angiotensinase C) is Increased in Obesity and Diabetes Mellitus and Related to Cardiovascular Dysfunction.
- Xu S, Lind L, Zhao L, Lindahl B, Venge P.SourceSections of Clinical Chemistry.
- Clinical chemistry.Clin Chem.2012 Apr 26. [Epub ahead of print]
- BACKGROUND: Prolylcarboxypeptidase (PRCP) (angiotensinase C) has 3 major targets, angiotensin II, prekallikrein, and α-melanocyte stimulating hormone(1-13). The truncation of the latter leads to loss in appetite regulation and obesity in experimental animals. The objectives of this study were to pu
- PMID 22539806
Japanese Journal
- Prekallikrein Deficiency in a Dog
- OKAWA Takumi,YANASE Takuma,SHIMOKAWA MIYAMA Takako,HIRAOKA Hiroko,BABA Kenji,TANI Kenji,OKUDA Masaru,MIZUNO Takuya
- Journal of Veterinary Medical Science advpub(0), 1008180313, 2010
- … Prekallikrein (PK) deficiency is an uncommon disorder in dogs. …
- NAID 130000307711
- 高齢になってから診断された先天性プレカリクレイン欠乏症の1症例
- 長屋 聡美,森下 英理子,高見 昭良,丸山 慶子,關谷 暁子,朝倉 英策,中尾 眞二,大竹 茂樹
- 日本老年医学会雑誌 46(4), 348-351, 2009-07-25
- NAID 10025638506
Related Links
- Prekallikrein (PK), also known as Fletcher factor, is an 85000 Mr serine protease that complexes with high-molecular-weight kininogen. PK is the precursor of plasma kallikrein, which is a serine protease that activates kinins. PK is cleaved to ...
- THE JOURNAL. 0 1986 by The American Society of Biological Chemists,. OF BIOLOGICAL CHEMISTRY. , Inc. Vol. 261, No. 1, Issue of January 5, pp. 324-327, 1986. Printed in U.S.A.. Human Plasma Prekallikrein. IMMUNdAFFINITY ...
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★リンクテーブル★
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- 英
- coagulation factor (Z), clotting factor, blood coagulation factor, blood coagulation factors
- 関
- ビタミンK
血液凝固因子
表
- 15種類ある
産生部位
臨床関連
-凝固因子
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- 英
- prekallikrein, PK
- 同
- フレッチャー因子 Fletcher factor
- 関
- 血液凝固因子