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)
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]
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]
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]
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リンク元 | 「血液凝固因子」「プレカリクレイン」 |
型 | 名称 | 同義語 | 分子量
(kDa) |
血漿中濃度
(μg/ml) |
半減期
(day) |
内因系のプロ酵素 | XII | Hageman因子 | 80 | 29 | 2 |
プレカリクレイン | Fletcher因子 | 88 | 45 | ||
XI | PTA | 160 | 4 | 2.5 | |
ビタミンK依存 | VII | proconvertin | 50 | 0.5 | 0.2 |
凝固プロ酵素 | IX | Christmas因子 | 57 | 4 | 1 |
X | Stuart因子 | 57 | 8 | 1.5 | |
II | プロトロンビン | 70 | 150 | 3 | |
補助因子 | III | 組織因子 | 0 | ||
血小板ホスホリピド | |||||
高分子量キニノゲン | 120 | 70 | |||
V | proaccelerin | 330 | 7 | 1.5 | |
VIII | 抗血友病因子 | 0.5 | |||
フィブリン堆積因子 | I | フィブリノゲン | 340 | 2500 | 4.5 |
XIII | フィブリン安定因子 | 320 | 8 | 7 | |
抑制物質 | アンチトロンビンIII | 58 | 150 | 2.5 |
凝固因子 | 慣用語・同義語 | ビタミンK依存 | 血液中半減期(hr) | 産生器官 | 染色体座乗部位 |
I | fibrinogen | 100-150 | 肝臓 | 4q26 | |
II | prothronbin | ○ | 50-80 | 肝臓 | 11p141-q12 |
III | tissue thromboplastin | 体の各組織 | 1pter-p21 | ||
IV | calcium ion | 肝臓 | |||
V | labile factor/proaccelerin | 24 | 肝臓 | 1q21-q25 | |
VII | stable factor/proconvertin | ○ | 6 | 肝臓 | 13q34 |
VIII | antihemophilic factor | 12 | 細網系? | xq28 | |
IX | Christmas factor | ○ | 24 | 肝臓 | xq26.3-q27.2 |
X | Stuart factor | ○ | 25-60 | 肝臓 | 13q34 |
XI | plasma thromboplastin antecedent | 40-80 | 肝臓 | 4q35 | |
XII | Hageman factor | 50-70 | 肝臓 | 5q33-qter | |
XIII | fibrin stabilizing factor | 150 | 肝臓 | 6p24-p21.3 | |
prekallikrein | Fletcher factor | 35 | 肝臓 | 4q35 | |
hight molecular weight kininogen | Fitzgerold factor | 150 | 肝臓 | 3q26-qter | |
von Willebrand factor | 24 | 血管内皮
血小板 糸球体 |
12pter-p12 |
-凝固因子
.