Mouse Genome Informatics
tg
    Tg(Vav1-NUP98/HOXD13)C1Apla/0
FVB/N-Tg(Vav1-NUP98/HOXD13)C1Apla
Key:
phenotype observed in females WTSI Wellcome Trust Sanger Institute
phenotype observed in males EuPh Europhenome
N normal phenotype
       
mortality/aging
• mice do not survive past 14 months, displaying a spectrum of hematologic malignancies at time of death, with individual mice exhibiting various leukemias or myelodysplastic syndrome (MDS)

hematopoietic system
• peripheral blood cytopenia and dysplasia are observed in mutants
• mutants show trend (not statistically significant) to increased mean cell volume
• circulating nucleated red blood cells are seen seen in peripheral blood smears from animals at 4 to 7 months
• seen in peripheral blood smears from animals at 4 to 7 months
• seen in peripheral blood smears from animals at 4 to 7 months
• seen in peripheral blood smears from animals at 4 to 7 months
• compared to littermate controls at 4 to 7 months
• hypersegmented neutrophils are seen in peripheral blood smears from animals at 4 to 7 months
• observed in contrast to littermate controls at 4 to 7 months
• bone marrow shows increased apoptosis compared to control littermates at 5 months
• dysplastic multinucleated erythroblasts are observed bone marrow of mutants at 5 months but not in controls; some erythroblasts with clefted nuclei are observed
• numbers of erythroblasts in bone marrow is higher than in controls
• bone marrow (from tibia or femur) is hypercellular (total number of nucleated cells) in some mice at 5 months
• dysplastic megakaryocytes with hypolobulated nuclei and increased nuclear-cytoplasmic ratios are observed in bone marrow of mutants but not in controls at 5 months
• giant platelets are seen in peripheral blood smears from animals at 4 to 7 months

tumorigenesis
• at time of death (around or before 14 months), some animals develop megakaryocytic leukemia, erythroid leukemia, undifferentiated leukemia, myeloid leukemia with maturation, and precursor-T lymphoblastic lymphoma/leukemia (pre-T LBL) or pre-malignant myelodysplastic syndrome (MDS)

immune system
• compared to littermate controls at 4 to 7 months
• hypersegmented neutrophils are seen in peripheral blood smears from animals at 4 to 7 months

Mouse Models of Human Disease
OMIM IDRef(s)
Myelodysplastic Syndrome; MDS 614286 J:107446