Abstract |
The study of coagulation factors has been rapidly advanced by studies performed in genetically engineered mouse strains. Investigation of factor IX (FIX) has benefited from excellent gene-deleted mouse models that recapitulate many of the features of human haemophilia B. Moreover, advanced positional cloning techniques and availability of technology to allow not only knock-out mice, but also knock-in and knock-down mice, provide new opportunities to observe genotype-phenotype and structure-function correlations regarding FIX, as well as the interaction of FIX with inflammatory, immune, and tissue repair systems. In this paper, available FIX knock-out mice and additional haemophilia B mouse models are reviewed specifically in regards to observations these models have facilitated concerning: factor IX gene expression and factor IX protein pharmacokinetics; the role of FIX in haemostasis, thrombosis and wound healing; insights into coagulation FIX arising out of gene therapy applications in haemophilia mouse models; immunology of tolerance or loss of tolerance of FIX and inhibitor antibody formation.
|
Authors | Paul E Monahan |
Journal | Thrombosis and haemostasis
(Thromb Haemost)
Vol. 100
Issue 4
Pg. 563-75
(Oct 2008)
ISSN: 0340-6245 [Print] Germany |
PMID | 18841277
(Publication Type: Journal Article, Research Support, N.I.H., Extramural)
|
Chemical References |
|
Topics |
- Animals
- Disease Models, Animal
- Factor IX
(genetics, immunology, metabolism)
- Genetic Therapy
- Hemophilia B
(genetics, immunology, therapy)
- Humans
- Mice
- Mice, Knockout
- Mice, Transgenic
|