Impaired clot retraction in factor XIII A subunit-deficient mice.

نویسندگان

  • Kohji Kasahara
  • Masayoshi Souri
  • Mizuho Kaneda
  • Toshiaki Miki
  • Naomasa Yamamoto
  • Akitada Ichinose
چکیده

Factor XIII (FXIII) is a plasma transglutaminase that cross-links fibrin monomers, alpha(2)-plasmin inhibitor, and so forth. Congenital FXIII deficiency causes lifelong bleeding symptoms. To understand the molecular pathology of FXIII deficiency in vivo, its knockout mice have been functionally analyzed. Because prolonged bleeding times, a sign of defective/abnormal primary hemostasis, were commonly observed in 2 separate lines of FXIII A subunit (FXIII-A) knockout mice, a possible role or roles of FXIII in platelet-related function was investigated in the present study. Although platelet aggregation induced by adenosine diphosphate or collagen was normal, clot retraction (CR) was lost in the platelet-rich plasma (PRP) of FXIII-A knockout mice. In contrast, there was no CR impairment in the PRP of tissue transglutaminase-knockout mice compared with that of wild-type mice. Furthermore, a transglutaminase inhibitor, cystamine, halted CR in the PRP of wild-type mice. These results indicate that the enzymatic activity of FXIII is necessary for CR, at least in mice.

برای دانلود رایگان متن کامل این مقاله و بیش از 32 میلیون مقاله دیگر ابتدا ثبت نام کنید

ثبت نام

اگر عضو سایت هستید لطفا وارد حساب کاربری خود شوید

منابع مشابه

Factor XIII deficiency: a review of literature

Coagulaon factor XIII gene, protein structure and funcon Coagulaon factor XIII (FXIII) is a tetrameric (FXIII- A2B2) pro-transglutaminase enzyme with an essenal role in the final stage of coagulaon cascade by cross linking the fibrin monomers and stabilizing the fibrin clot. Congenital FXIII deficiency is a rare bleeding disorder, with an autosomal recessive trait inheritance, and a fre...

متن کامل

Acceleration of fibrinolysis by the N-terminal peptide of alpha 2-plasmin inhibitor.

When blood plasma containing the NH2-terminal 12-residue peptide (N-peptide) of alpha 2-plasmin inhibitor (alpha 2PI; alpha 2-antiplasmin) was clotted in the presence of calcium ions, the N-peptide and alpha 2PI were cross-linked to fibrin by activated coagulation factor XIII. The amount of N-peptide cross-linked to fibrin was proportional to the concentration of N-peptide present in plasma. On...

متن کامل

Regular Article PLATELETS AND THROMBOPOIESIS Clot retraction is mediated by factor XIII-dependent fibrin-aIIbb3- myosin axis in platelet sphingomyelin-rich membrane rafts

Clot retraction is mediated by factor XIII-dependent fibrin-aIIbb3myosin axis in platelet sphingomyelin-rich membrane rafts Kohji Kasahara, Mizuho Kaneda, Toshiaki Miki, Kazuko Iida, Naoko Sekino-Suzuki, Ikuo Kawashima, Hidenori Suzuki, Motoyuki Shimonaka, Morio Arai, Yoshiko Ohno-Iwashita, Soichi Kojima, Mitsuhiro Abe, Toshihide Kobayashi, Toshiro Okazaki, Masayoshi Souri, Akitada Ichinose, an...

متن کامل

EMILIN2 Regulates Platelet Activation, Thrombus Formation, and Clot Retraction

Thrombosis, like other cardiovascular diseases, has a strong genetic component, with largely unknown determinants. EMILIN2, Elastin Microfibril Interface Located Protein2, was identified as a candidate gene for thrombosis in mouse and human quantitative trait loci studies. EMILIN2 is expressed during cardiovascular development, on cardiac stem cells, and in heart tissue in animal models of hear...

متن کامل

ذخیره در منابع من


  با ذخیره ی این منبع در منابع من، دسترسی به آن را برای استفاده های بعدی آسان تر کنید

عنوان ژورنال:
  • Blood

دوره 115 6  شماره 

صفحات  -

تاریخ انتشار 2010