ArticlePloS one2012
Porcine model of hemophilia A.
Article in PloS one, 2012. The graph could read no effect estimate from its abstract, so it casts no vote on the map. Cited by 19 papers.
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Who cites it
19 citing papers in PubMed, 37 citations in OpenAlex.
- From farm to lab: gene-edited sheep transforming bone research.JBMR plus · 2026Review
- In Vivo Porcine Model of Acute Iliocaval Deep Vein Thrombosis.Journal of endovascular therapy : an official journal of the International Society of Endovascular Specialists · 2025Article
- Generation of Mouse Model of Hemophilia A by Introducing Novel Mutations, Using CRISPR/Nickase Gene Targeting System.Cell journal · 2023Article
- Development of alternative gene transfer techniques forRegenerative therapy · 2021Article
- Advances in gene therapy for hemophilia: basis, current status, and future perspectives.International journal of hematology · 2020Review
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- Embryo production by intracytoplasmic injection of sperm retrieved from Meishan neonatal testicular tissue cryopreserved and grafted into nude mice.Animal science journal = Nihon chikusan Gakkaiho · 2019Article
- Safety of intra-articular transplantation of lentivirally transduced mesenchymal stromal cells for haemophilic arthropathy in a non-human primate.International journal of hematology · 2018Article
- Modeling lethal X-linked genetic disorders in pigs with ensured fertility.Proceedings of the National Academy of Sciences of the United States of America · 2018Article
- Establishment of a strain of haemophilia-A pigs by xenografting of foetal testicular tissue from neonatally moribund cloned pigs.Scientific reports · 2017Article
- Genetically engineered livestock for biomedical models.Transgenic research · 2016Review
- Characterization of a genetically engineered mouse model of hemophilia A with complete deletion of the F8 gene.Journal of thrombosis and haemostasis : JTH · 2016Article
- Current animal models of hemophilia: the state of the art.Thrombosis journal · 2016Review
- Contemporary Animal Models For Human Gene Therapy Applications.Current gene therapy · 2015Review
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- Lessons Learned from Animal Models of Inherited Bleeding Disorders.Hematology education. European Hematology Association. Congress. Education Program · 2014Article
- Genetically modified pigs to model human diseases.Journal of applied genetics · 2014Review
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Corrections and comments
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Authors and funding
16 authors at 2 institutions in 1 country.
Funding
No grant is acknowledged in the PubMed record.
Abstract
Hemophilia A is a common X chromosome-linked genetic bleeding disorder caused by abnormalities in the coagulation factor VIII gene (F8). Hemophilia A patients suffer from a bleeding diathesis, such as life-threatening bleeding in the brain and harmful bleeding in joints and muscles. Because it could potentially be cured by gene therapy, subhuman animal models have been sought. Current mouse hemophilia A models generated by gene targeting of the F8 have difficulties to extrapolate human disease due to differences in the coagulation and immune systems between mice and humans. Here, we generated a porcine model of hemophilia A by nuclear transfer cloning from F8-targeted fibroblasts. The hemophilia A pigs showed a severe bleeding tendency upon birth, similar to human severe hemophiliacs, but in contrast to hemophilia A mice which rarely bleed under standard breed conditions. Infusion of human factor VIII was effective in stopping bleeding and reducing the bleeding frequency of a hemophilia A piglet but was blocked by the inhibitor against human factor VIII. These data suggest that the hemophilia A pig is a severe hemophilia A animal model for studying not only hemophilia A gene therapy but also the next generation recombinant coagulation factors, such as recombinant factor VIII variants with a slower clearance rate.
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Registered trials
Read under generation 80e0d062 · epoch 390. Bibliography from PubMed, PubMed Central and OpenAlex; grants from NIH RePORTER; trial links from ClinicalTrials.gov; estimates, votes and beliefs from the OpenQuestion graph.