SEARCH:   Advanced

This Article Full Text Full Text (PDF) Alert me when this article is cited Alert me if a correction is posted Citation Map Services Email this article to a friend Similar articles in this journal Similar articles in PubMed Alert me to new issues of the journal Download to citation manager Rights and Permissions Citing Articles Citing Articles via HighWire Citing Articles via CrossRef Citing Articles via Google Scholar Google Scholar Articles by Lin, H.-F. Articles by Stafford, D. W. Search for Related Content PubMed PubMed Citation Articles by Lin, H.-F. Articles by Stafford, D. W. Related Collections Hemostasis, Thrombosis, and Vascular Biology Social Bookmarking                   What's this?  Previous Article  |  Table of Contents  |  Next Article  A Coagulation Factor IX-Deficient Mouse Model for Human Hemophilia B Hui-Feng Lin, Nobuyo Maeda, Oliver Smithies, David L. Straight, and Darrel W. Stafford From the Department of Biology, Department of Pathology and Laboratory Medicine, and Center for Thrombosis and Hemostasis, University of North Carolina at Chapel Hill, Chapel Hill, NC. Coagulation factor IX deficiency causes hemophilia B in humans. We have used gene targeting to develop a coagulation factor IX-deficient (factor IX-knockout) mouse strain. Mouse embryonic stem (ES) cells were targeted by a socket-containing vector that replaces the promoter through exon 3 of the factor IX gene by neoHPRT, which is a functional neo gene plus a partially deleted hypoxanthine phosphoribosyl transferase minigene. Chimeric mice generated using these socket-containing ES cells transmitted the targeted factor IX gene to their female offspring. Male offspring from these females were characterized and shown to exhibit a phenotype similar to hemophilia B. This factor IX-deficient mouse strain will be useful for studying gene therapy methods and structure-function relationships of recombinant factor IX proteins in vivo. Blood, Vol. 90 No. 10 (November 15), 1997: pp. 3962-3966 © 1997 by The American Society of Hematology. CiteULike    Connotea    Del.icio.us    Digg    Reddit    Technorati    What's this? This article has been cited by other articles: J. C McCann and B. N Ames Vitamin K, an example of triage theory: is micronutrient inadequacy linked to diseases of aging? Am. J. Clinical Nutrition, October 1, 2009; 90(4): 889 - 907. [Abstract] [Full Text] [PDF] K. Kalippke, S. Werwitzke, M. von Hornung, R. Mischke, A. Ganser, and A. Tiede DNA analysis from stool samples: a fast and reliable method avoiding invasive sampling methods in mouse models of bleeding disorders Lab Anim, October 1, 2009; 43(4): 390 - 393. [Abstract] [Full Text] [PDF] J. Sun, N. Hakobyan, L. A. Valentino, B. L. Feldman, R. J. Samulski, and P. E. Monahan Intraarticular factor IX protein or gene replacement protects against development of hemophilic synovitis in the absence of circulating factor IX Blood, December 1, 2008; 112(12): 4532 - 4541. [Abstract] [Full Text] [PDF] Y. Buyue, H. C. Whinna, and J. P. Sheehan The heparin-binding exosite of factor IXa is a critical regulator of plasma thrombin generation and venous thrombosis Blood, October 15, 2008; 112(8): 3234 - 3241. [Abstract] [Full Text] [PDF] A. G. McDonald, K. Yang, H. R. Roberts, D. M. Monroe, and M. Hoffman Perivascular tissue factor is down-regulated following cutaneous wounding: implications for bleeding in hemophilia Blood, February 15, 2008; 111(4): 2046 - 2048. [Abstract] [Full Text] [PDF] S. Merkulov, W.-M. Zhang, A. A. Komar, A. H. Schmaier, E. Barnes, Y. Zhou, X. Lu, T. Iwaki, F. J. Castellino, G. Luo, et al. Deletion of murine kininogen gene 1 (mKng1) causes loss of plasma kininogen and delays thrombosis Blood, February 1, 2008; 111(3): 1274 - 1281. [Abstract] [Full Text] [PDF] D. Gailani and T. Renne Intrinsic Pathway of Coagulation and Arterial Thrombosis Arterioscler Thromb Vasc Biol, December 1, 2007; 27(12): 2507 - 2513. [Abstract] [Full Text] [PDF] M. Hoffman, A. Harger, A. Lenkowski, U. Hedner, H. R. Roberts, and D. M. Monroe Cutaneous wound healing is impaired in hemophilia B Blood, November 1, 2006; 108(9): 3053 - 3060. [Abstract] [Full Text] [PDF] O. Cao, E. Armstrong, A. Schlachterman, L. Wang, D. K. Okita, B. Conti-Fine, K. A. High, and R. W. Herzog Immune deviation by mucosal antigen administration suppresses gene-transfer-induced inhibitor formation to factor IX Blood, July 15, 2006; 108(2): 480 - 486. [Abstract] [Full Text] [PDF] N. Mackman Tissue-Specific Hemostasis in Mice Arterioscler Thromb Vasc Biol, November 1, 2005; 25(11): 2273 - 2281. [Abstract] [Full Text] [PDF] Q. Cheng, Y. Zhao, W. E. Lawson, V. V. Polosukhin, J. E. Johnson, T. S. Blackwell, and D. Gailani The effects of intrinsic pathway protease deficiencies on plasminogen-deficient mice Blood, November 1, 2005; 106(9): 3055 - 3057. [Abstract] [Full Text] [PDF] D. M. Shayakhmetov, A. Gaggar, S. Ni, Z.-Y. Li, and A. Lieber Adenovirus Binding to Blood Factors Results in Liver Cell Infection and Hepatotoxicity J. Virol., June 15, 2005; 79(12): 7478 - 7491. [Abstract] [Full Text] [PDF] J. H. Fair, B. A. Cairns, M. A. LaPaglia, M. Caballero, W. A. Pleasant, S. Hatada, H.-s. Kim, T. Gui, L. Pevny, A. A. Meyer, et al. Correction of factor IX deficiency in mice by embryonic stem cells differentiated in vitro PNAS, February 22, 2005; 102(8): 2958 - 2963. [Abstract] [Full Text] [PDF] D. Lillicrap Testing novel hemophilia therapies: of mice and men Blood, November 1, 2004; 104(9): 2614 - 2615. [Full Text] [PDF] D. E. Sabatino, E. Armstrong, S. Edmonson, Y.-L. Liu, M. Pleimes, J. Schuettrumpf, J. Fitzgerald, R. W. Herzog, V. R. Arruda, and K. A. High Novel hemophilia B mouse models exhibiting a range of mutations in the Factor IX gene Blood, November 1, 2004; 104(9): 2767 - 2774. [Abstract] [Full Text] [PDF] D.-Y. Jin, T.-P. Zhang, T. Gui, D. W. Stafford, and P. E. Monahan Creation of a mouse expressing defective human factor IX Blood, September 15, 2004; 104(6): 1733 - 1739. [Abstract] [Full Text] [PDF] N. Mackman Role of Tissue Factor in Hemostasis, Thrombosis, and Vascular Development Arterioscler Thromb Vasc Biol, June 1, 2004; 24(6): 1015 - 1022. [Abstract] [Full Text] [PDF] V. R. Arruda, J. Schuettrumpf, R. W. Herzog, T. C. Nichols, N. Robinson, Y. Lotfi, F. Mingozzi, W. Xiao, L. B. Couto, and K. A. High Safety and efficacy of factor IX gene transfer to skeletal muscle in murine and canine hemophilia B models by adeno-associated viral vector serotype 1 Blood, January 1, 2004; 103(1): 85 - 92. [Abstract] [Full Text] [PDF] J. Zhang, L. Xu, M. E. Haskins, and K. Parker Ponder Neonatal gene transfer with a retroviral vector results in tolerance to human factor IX in mice and dogs Blood, January 1, 2004; 103(1): 143 - 151. [Abstract] [Full Text] [PDF] M. Moser and C. Patterson Thrombin and Vascular Development: A Sticky Subject Arterioscler Thromb Vasc Biol, June 1, 2003; 23(6): 922 - 930. [Abstract] [Full Text] [PDF] L. Xu, C. Gao, M. S. Sands, S.-R. Cai, T. C. Nichols, D. A. Bellinger, R. A. Raymer, S. McCorquodale, and K. P. Ponder Neonatal or hepatocyte growth factor-potentiated adult gene therapy with a retroviral vector results in therapeutic levels of canine factor IX for hemophilia B Blood, May 15, 2003; 101(10): 3924 - 3932. [Abstract] [Full Text] [PDF] C. S. Manno, A. J. Chew, S. Hutchison, P. J. Larson, R. W. Herzog, V. R. Arruda, S. J. Tai, M. V. Ragni, A. Thompson, M. Ozelo, et al. AAV-mediated factor IX gene transfer to skeletal muscle in patients with severe hemophilia B Blood, April 15, 2003; 101(8): 2963 - 2972. [Abstract] [Full Text] [PDF] A. Ameri, S. Kurachi, K. Sueishi, M. Kuwahara, and K. Kurachi Myocardial fibrosis in mice with overexpression of human blood coagulation factor IX Blood, March 1, 2003; 101(5): 1871 - 1873. [Abstract] [Full Text] [PDF] R. Pawlinski, A. Fernandes, B. Kehrle, B. Pedersen, G. Parry, J. Erlich, R. Pyo, D. Gutstein, J. Zhang, F. Castellino, et al. Tissue factor deficiency causes cardiac fibrosis and left ventricular dysfunction PNAS, November 26, 2002; 99(24): 15333 - 15338. [Abstract] [Full Text] [PDF] T. Gui, H.-F. Lin, D.-Y. Jin, M. Hoffman, D. L. Straight, H. R. Roberts, and D. W. Stafford Circulating and binding characteristics of wild-type factor IX and certain Gla domain mutants in vivo Blood, June 17, 2002; 100(1): 153 - 158. [Abstract] [Full Text] [PDF] A. Ehrhardt and M. A. Kay A new adenoviral helper-dependent vector results in long-term therapeutic levels of human coagulation factor IX at low doses in vivo Blood, May 13, 2002; 99(11): 3923 - 3930. [Abstract] [Full Text] [PDF] J. D. Mount, R. W. Herzog, D. M. Tillson, S. A. Goodman, N. Robinson, M. L. McCleland, D. Bellinger, T. C. Nichols, V. R. Arruda, C. D. Lothrop Jr, et al. Sustained phenotypic correction of hemophilia B dogs with a factor IX null mutation by liver-directed gene therapy Blood, April 15, 2002; 99(8): 2670 - 2676. [Abstract] [Full Text] [PDF] E. D. Rosen, S. Raymond, A. Zollman, F. Noria, M. Sandoval-Cooper, A. Shulman, J. L. Merz, and F. J. Castellino Laser-Induced Noninvasive Vascular Injury Models in Mice Generate Platelet- and Coagulation-Dependent Thrombi Am. J. Pathol., May 1, 2001; 158(5): 1613 - 1622. [Abstract] [Full Text] [PDF] K. A. High Gene Transfer as an Approach to Treating Hemophilia Circ. Res., February 2, 2001; 88(2): 137 - 144. [Abstract] [Full Text] [PDF] B. D. Car and V. M. Eng Special Considerations in the Evaluation of the Hematology and Hemostasis of Mutant Mice Vet. Pathol., January 1, 2001; 38(1): 20 - 30. [Abstract] [Full Text] [PDF] M. A. Kay and K. High Gene therapy for the hemophilias PNAS, August 31, 1999; 96(18): 9973 - 9975. [Full Text] [PDF] D. W. Russell and M. A. Kay Adeno-Associated Virus Vectors and Hematology Blood, August 1, 1999; 94(3): 864 - 874. [Full Text] [PDF] L. Wang, K. Takabe, S. M. Bidlingmaier, C. R. Ill, and I. M. Verma Sustained correction of bleeding disorder in hemophilia B mice by gene therapy PNAS, March 30, 1999; 96(7): 3906 - 3910. [Abstract] [Full Text] [PDF] J. N. Lozier, M. E. Metzger, R. E. Donahue, and R. A. Morgan The Rhesus Macaque as an Animal Model for Hemophilia B Gene Therapy Blood, March 15, 1999; 93(6): 1875 - 1881. [Abstract] [Full Text] [PDF] R. K. Kundu, F. Sangiorgi, L.-Y. Wu, K. Kurachi, W. F. Anderson, R. Maxson, and E. M. Gordon Targeted Inactivation of the Coagulation Factor IX Gene Causes Hemophilia B in Mice Blood, July 1, 1998; 92(1): 168 - 174. [Abstract] [Full Text] [PDF] S.-H. Kung, J. Nathan Hagstrom, D. Cass, S. Jen Tai, H.-F. Lin, D. W. Stafford, and K. A. High Human Factor IX Corrects the Bleeding Diathesis of Mice With Hemophilia B Blood, February 1, 1998; 91(3): 784 - 790. [Abstract] [Full Text] [PDF]

	Copyright © 1997 by American Society of Hematology         Online ISSN: 1528-0020