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Measurement of Factor V Activity in Human Plasma Using a Microplate Coagulation Assay
Published on: September 9, 2012
Combined factor VII/protein C deficiency results in intrauterine coagulopathy in mice
J C Chan1, I Cornelissen, D Collen
1W.M. Keck Center for Transgene Research and the Department of Chemistry and Biochemistry, University of Notre Dame, Notre Dame, Indiana 46556, USA.
The Journal of Clinical Investigation
|April 5, 2000
Summary
Mice lacking both protein C and factor VII genes showed severe coagulopathy and embryonic mortality. Loss of factor VII exacerbates protein C deficiency, impacting blood clotting in developing embryos.
Area of Science:
- Hematology
- Developmental Biology
- Genetics
Background:
- Protein C deficiency (PC(-/-)) in embryos causes coagulopathy.
- Factor VII (FVII) is crucial for blood coagulation.
- The interaction between PC and FVII deficiencies in development is not well understood.
Purpose of the Study:
- To investigate the impact of combined FVII and PC gene loss on embryonic coagulopathy.
- To determine if FVII deficiency exacerbates the phenotype of PC deficiency.
Main Methods:
- Crossed mice doubly heterozygous for FVII and PC genes.
- Analyzed 9 predicted genotypic combinations in offspring.
- Assessed embryonic phenotypes, coagulopathy, hemorrhage, edema, and mortality.
Main Results:
- FVII(-/-)/PC(-/-) embryos exhibited severe intra- and extravascular coagulopathy, hemorrhage, edema, and perinatal mortality.
- FVII(+/-)/PC(-/-) embryos showed a less severe phenotype, indicating a gene dosage effect.
- Fibrin presence in FVII-deficient embryos suggests alternative clot-generating pathways.
Conclusions:
- Combined FVII and PC deficiency leads to a severe, previously unobserved embryonic coagulopathy.
- Factor VII deficiency significantly worsens protein C deficiency, likely due to increased factor Xa and thrombin generation.
- Embryonic clot formation can occur independently of the FVII-dependent pathway.
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