Phenotypes associated with this allele

• Allele Symbol: Tg(PML-RARA)556Kog
• Allele Name: transgene insertion 556, Scott Kogan
• Allele ID: MGI:3033300
• Summary: 3 genotypes

Jump to	Allelic Composition	Genetic Background	Genotype ID
cx1	Pml^tm1.1Ews/Pml^tm1.1Ews Tg(PML-RARA)556Kog/0	FVB.Cg-Pml^tm1.1Ews Tg(PML-RARA)556Kog	MGI:6195067
tg2	Tg(PML-RARA)556Kog/0	FVB/N-Tg(PML-RARA)556Kog	MGI:6195072
tg3	Tg(PML-RARA)556Kog/?	involves: FVB/N	MGI:3815135

Genotype
MGI:6195067

cx1

• Allelic Composition: Pml^tm1.1Ews/Pml^tm1.1Ews; Tg(PML-RARA)556Kog/0
• Genetic Background: FVB.Cg-Pml^tm1.1Ews Tg(PML-RARA)556Kog

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

cellular

abnormal double-strand DNA break repair ( J:262172 )

• primary MEFs show a defect in nonhomologous end-joining (NHEJ)

hematopoietic system

enlarged spleen ( J:262172 )

anemia ( J:262172 )

thrombocytosis ( J:262172 )

increased leukocyte cell number ( J:262172 )

• leukemia is characterized by hyperleukocytosis

homeostasis/metabolism

abnormal double-strand DNA break repair ( J:262172 )

• primary MEFs show a defect in nonhomologous end-joining (NHEJ)

immune system

enlarged spleen ( J:262172 )

increased leukocyte cell number ( J:262172 )

• leukemia is characterized by hyperleukocytosis

neoplasm

increased acute promyelocytic leukemia incidence ( J:262172 )

• mice develop acute promyelocytic leukemia spontaneously with a frequency of 13.8% at 18 months of age

• leukemias are characterized by hyperleukocytosis, anemia, and thrombocytosis

growth/size/body

enlarged spleen ( J:262172 )

Mouse Models of Human Disease		DO ID	OMIM ID(s)	Ref(s)
acute promyelocytic leukemia		DOID:0060318	OMIM:612376	J:262172

Genotype
MGI:6195072

tg2

• Allelic Composition: Tg(PML-RARA)556Kog/0
• Genetic Background: FVB/N-Tg(PML-RARA)556Kog

hematopoietic system

enlarged spleen ( J:262172 )

anemia ( J:262172 )

thrombocytosis ( J:262172 )

increased leukocyte cell number ( J:262172 )

• leukemia is characterized by hyperleukocytosis

immune system

enlarged spleen ( J:262172 )

increased leukocyte cell number ( J:262172 )

• leukemia is characterized by hyperleukocytosis

neoplasm

increased acute promyelocytic leukemia incidence ( J:262172 )

• mice develop acute promyelocytic leukemia spontaneously with a frequency of 6.8% at 18 months of age

• leukemias are characterized by hyperleukocytosis, anemia, and thrombocytosis

growth/size/body

enlarged spleen ( J:262172 )

Genotype
MGI:3815135

tg3

• Allelic Composition: Tg(PML-RARA)556Kog/?
• Genetic Background: involves: FVB/N

neoplasm

increased skin papilloma incidence ( J:39327 )

• epidermal papillomatosis

increased acute promyelocytic leukemia incidence ( J:39327 )

• mutants develop acute promyelocytic leukemia between 3 and 9 months of age, with a median age of 174 days

• mixed hematopoiesis of normal bone marrow is completely replaced by leukemic cells, splenic and lymphatic architecture is effaced and leukemia extends throughout the hepatic parenchyma

• both preleukemic state and the leukemia can be transplanted to wild-type mice and the preleukemia progresses to acute promyelocytic leukemia

immune system

abnormal neutrophil differentiation ( J:39327 )

• impaired neutrophil maturation early in life

hematopoietic system

abnormal neutrophil differentiation ( J:39327 )

• impaired neutrophil maturation early in life

anemia ( J:39327 )

thrombocytopenia ( J:39327 )

cardiovascular system

hemorrhage ( J:39327 )

• some leukemic mutants hemorrhage spontaneously

homeostasis/metabolism

abnormal blood coagulation ( J:39327 )

• leukemic mutants are abnormally susceptible to bleeding after injections

abnormal lung thrombosis ( J:39327 )

• some mutants exhibit fibrin thrombi in pulmonary blood vessels but not in other organs

integument

increased skin papilloma incidence ( J:39327 )

• epidermal papillomatosis

cellular

abnormal neutrophil differentiation ( J:39327 )

• impaired neutrophil maturation early in life

Mouse Models of Human Disease		DO ID	OMIM ID(s)	Ref(s)
acute promyelocytic leukemia		DOID:0060318	OMIM:612376	J:39327