Phenotypes associated with this allele

• Allele Symbol: Tg(CTSG-PML/RARA)#Ppp
• Allele Name: transgene insertion, Pier Paolo Pandolfi
• Allele ID: MGI:3040508
• Summary: 1 genotype

Jump to	Allelic Composition	Genetic Background	Genotype ID
tg1	Tg(CTSG-PML/RARA)#Ppp/0	Not Specified	MGI:5514263

Genotype
MGI:5514263

tg1

• Allelic Composition: Tg(CTSG-PML/RARA)#Ppp/0
• Genetic Background: Not Specified

• ♀: phenotype observed in females
• ♂: phenotype observed in males
• N: normal phenotype

hematopoietic system

abnormal definitive hematopoiesis ( J:40373 )

• clonogenic capacity of hematopoietic precursors is reduced, however, the number of clusters in bone marrow and spleen cultures is increased compared to wild-type cultures

abnormal myelopoiesis ( J:40373 , J:78201 )

• disruption of myeloid hematopoiesis, with mutants developing a myeloproliferative disorder by 12 months of age, characterized by accumulation of myeloid precursors, including promyelocytes, in the bone marrow, that infiltrate the spleen, the lymph nodes, and the thymus (J:40373)

• accumulation of c-Kit+ cells in the spleen and bone marrow indicating impaired myeloid differentiation (J:78201)

decreased erythroid progenitor cell number ( J:40373 )

• clonogenic capacity of hematopoietic precursors is reduced, particularly the number of BFU-E

increased leukocyte cell number ( J:40373 )

• mutants with leukemia show an increase in white blood cell counts in the peripheral blood

increased granulocyte number ( J:40373 )

• an increase in granulocyte/lymphocyte ratio is seen in the peripheral blood of some mutants

immune system

abnormal myelopoiesis ( J:40373 , J:78201 )

• disruption of myeloid hematopoiesis, with mutants developing a myeloproliferative disorder by 12 months of age, characterized by accumulation of myeloid precursors, including promyelocytes, in the bone marrow, that infiltrate the spleen, the lymph nodes, and the thymus (J:40373)

• accumulation of c-Kit+ cells in the spleen and bone marrow indicating impaired myeloid differentiation (J:78201)

increased leukocyte cell number ( J:40373 )

• mutants with leukemia show an increase in white blood cell counts in the peripheral blood

increased granulocyte number ( J:40373 )

• an increase in granulocyte/lymphocyte ratio is seen in the peripheral blood of some mutants

mortality/aging

premature death ( J:40373 )

• in mutants that develop leukemia

neoplasm

increased leukemia incidence ( J:40373 , J:78201 )

• about 10% of mutants develop overt acute leukemia after they reach 1 year of age (J:40373)

• retinoic acid markedly increases the terminal differentiation of leukemic cells to a larger extent than wild-type hematopoietic precursors, indicating an increased sensitivity of leukemic blasts to the differentiating action of retinoic acid (J:40373)

• 25% of mutants develop leukemia between 6 months to 1.5 years of age (J:78201)

• treatment of mice with retinoic acid results in remission of leukemia by week three of treatment, prolonging survival and inducing hematopoietic differentiation, however, mice relapse shortly after remission and die within 8 weeks of treatment (J:78201)

increased acute promyelocytic leukemia incidence ( J:40373 , J:78201 )

• leukemia is characterized by accumulation of promyelocytes in the bone marrow, peripheral blood, and spleen, indicative of acute promyelocytic leukemia (J:40373)

• some promyelocytes in the leukemia show the presence of pathognomonic cytoplasmic Auer's bodies (J:40373)

• leukemic cellular proliferation retains the ability to terminally differentiate toward mature granulocytes (J:40373)