Murine models of eosinophilic leukemia: a model of FIP1L1-PDGFRα initiated chronic eosinophilic leukemia/systemic mastocytosis.

Abstract

Chronic eosinophilic leukemia (CEL) was distinguished from hypereosinophilic syndrome (HES) in the 2001 World Health Organization (WHO) criteria. Subsequently, the FIP1L1-PDGFRα (F/P) fusion tyrosine kinase was identified in patients with HES and found to be the most common clonal defect in CEL and the second most frequent mutation in systemic mastocytosis (SM). Introduction of F/P into bone marrow hematopoietic stem cells and progenitors has been used to establish murine models of F/P-myeloproliferative neoplasm and F/P-CEL. IL-5 overexpression and introduction of F/P is required to develop murine CEL. This F/P-CEL model is thought to be an accurate model of the clinical disease. Here we describe the method of F/P-CEL/SM model development and assessment.

• Authors: Yoshiyuki Yamada, Jose A Cancelas, Marc E Rothenberg
• Journal: Methods in molecular biology (Clifton, N.J.) (Methods Mol Biol) Vol. 1178 Pg. 309-20 ( 2014) ISSN: 1940-6029 [Electronic] United States
• PMID: 24986627 (Publication Type: Journal Article, Research Support, Non-U.S. Gov't)

Chemical References

• Receptor, Platelet-Derived Growth Factor alpha

Topics

• Animals
• Disease Models, Animal
• Hematopoietic Stem Cells (cytology)
• Hypereosinophilic Syndrome (immunology, metabolism)
• Leukemia
• Mastocytosis, Systemic (immunology, metabolism)
• Mice
• Receptor, Platelet-Derived Growth Factor alpha (metabolism)