Myeloproliferative Neoplasm Animal Models
| Autor: | Benjamin L. Ebert, Ann Mullally, Kristina Brumme, Steven W. Lane |
|---|---|
| Rok vydání: | 2012 |
| Předmět: |
Pathology
medicine.medical_specialty Myeloproliferative Disorders Transgene food and beverages Hematology Biology medicine.disease Article Transplantation Disease Models Animal Mice Haematopoiesis medicine.anatomical_structure Oncology medicine Animals Humans Bone marrow Progenitor cell Myelofibrosis Myeloproliferative neoplasm |
| Zdroj: | Hematology/Oncology Clinics of North America. 26:1065-1081 |
| ISSN: | 0889-8588 |
| DOI: | 10.1016/j.hoc.2012.07.007 |
| Popis: | Myeloproliferative neoplasm (MPN) animal models accurately re-capitulate human disease in mice and have been an important tool for the study of MPN biology and therapy. Transplantation of BCR-ABL transduced bone marrow cells into irradiated syngeneic mice established the field of MPN animal modeling and the retroviral bone marrow transplantation (BMT) assay has been used extensively since. Genetically engineered MPN animal models have enabled detailed characterization of the effects of specific MPN associated genetic abnormalities on the hematopoietic stem and progenitor cell (HSPC) compartment and xenograft models have allowed the study of primary human MPN-propagating cells in vivo. All models have facilitated the pre-clinical development of MPN therapies. JAK2V617F, the most common molecular abnormality in BCR-ABL negative MPN, has been extensively studied using retroviral, transgenic, knock-in and xenograft models. MPN animal models have also been used to investigate additional genetic lesions found in human MPN and to evaluate the bone marrow microenvironment in these diseases. Finally, several genetic lesions, although not common, somatically mutated drivers of MPN in humans induce a MPN phenotype in mice. Future uses for MPN animal models will include modeling compound genetic lesions in MPN and studying myelofibrotic transformation. |
| Databáze: | OpenAIRE |
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