Literature DB >> 7959353

Factor VIII in blood plasma of haemophilic sheep: analysis of clotting time-plasma dilution curves.

S Neuenschwander1, V Pliska.   

Abstract

The sheep haemophilia A, recently described in a flock of White Alpine sheep, is probably caused by the production of a less potent mutant of factor VIII (F-VIII). Other potential mechanisms, such as drastic reduction (or lack) of F-VIII production, or occurrence of an F-VIII inhibitor, are less likely. This conclusion was based on the analysis of plasma dilution-clotting effect relationships in normal female (anestrous, non-pregnant) sheep, female carriers of haemophilia A, and afflicted male sheep. A response model underlying the analysis was suggested, its validity tested, and a mathematical analysis suitable for routine evaluation procedure recommended.

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Year:  1994        PMID: 7959353     DOI: 10.1159/000217077

Source DB:  PubMed          Journal:  Haemostasis        ISSN: 0301-0147


  7 in total

Review 1.  Animal models of hemophilia and related bleeding disorders.

Authors:  Jay N Lozier; Timothy C Nichols
Journal:  Semin Hematol       Date:  2013-04       Impact factor: 3.851

2.  Clinical and molecular characterization of a re-established line of sheep exhibiting hemophilia A.

Authors:  C D Porada; C Sanada; C R Long; J A Wood; J Desai; N Frederick; L Millsap; C Bormann; S L Menges; C Hanna; G Flores-Foxworth; T Shin; M E Westhusin; W Liu; H Glimp; E D Zanjani; J N Lozier; V Pliska; G Stranzinger; H Joerg; D C Kraemer; G Almeida-Porada
Journal:  J Thromb Haemost       Date:  2009-11-23       Impact factor: 5.824

3.  Treatment of Hemophilia A in Utero and Postnatally using Sheep as a Model for Cell and Gene Delivery.

Authors:  Christopher D Porada; Graça Almeida-Porada
Journal:  J Genet Syndr Gene Ther       Date:  2012-05-25

Review 4.  Hemophilia A: an ideal disease to correct in utero.

Authors:  Christopher D Porada; Christopher Rodman; Glicerio Ignacio; Anthony Atala; Graça Almeida-Porada
Journal:  Front Pharmacol       Date:  2014-12-11       Impact factor: 5.810

5.  In vitro and In vivo Model Systems for Hemophilia A Gene Therapy.

Authors:  Jianhua Mao; Xiaodong Xi; Philipp Kapranov; Biao Dong; Jenni Firrman; Ruian Xu; Weidong Xiao
Journal:  J Genet Syndr Gene Ther       Date:  2013-01-17

Review 6.  Current animal models of hemophilia: the state of the art.

Authors:  Ching-Tzu Yen; Meng-Ni Fan; Yung-Li Yang; Sheng-Chieh Chou; I-Shing Yu; Shu-Wha Lin
Journal:  Thromb J       Date:  2016-10-04

Review 7.  In utero stem cell transplantation and gene therapy: rationale, history, and recent advances toward clinical application.

Authors:  Graça Almeida-Porada; Anthony Atala; Christopher D Porada
Journal:  Mol Ther Methods Clin Dev       Date:  2016-03-30       Impact factor: 6.698

  7 in total

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