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Phenotypes associated with this allele
Allele Symbol
Allele Name
Allele ID
Tg(CTSG-ZBTB16/RARA)#Ppp
transgene insertion, Pier Paolo Pandolfi
MGI:3527937
Summary 3 genotypes
Jump to Allelic Composition Genetic Background Genotype ID
cx1
Tg(CTSG-ZBTB16/RARA)#Ppp/0
Zbtb16tm1Ppp/Zbtb16tm1Ppp
involves: 129S1/Sv MGI:5514344
cx2
Tg(CTSG-RARA/ZBTB16)#Ppp/0
Tg(CTSG-ZBTB16/RARA)#Ppp/0
Not Specified MGI:5514339
tg3
Tg(CTSG-ZBTB16/RARA)#Ppp/0 Not Specified MGI:5514273


Genotype
MGI:5514344
cx1
Allelic
Composition
Tg(CTSG-ZBTB16/RARA)#Ppp/0
Zbtb16tm1Ppp/Zbtb16tm1Ppp
Genetic
Background
involves: 129S1/Sv
Find Mice Using the International Mouse Strain Resource (IMSR)
Mouse lines carrying:
Tg(CTSG-ZBTB16/RARA)#Ppp mutation (0 available)
Zbtb16tm1Ppp mutation (0 available); any Zbtb16 mutation (19 available)
phenotype observed in females
phenotype observed in males
N normal phenotype
neoplasm
• develop leukemia after 6 months of age characterized by an accumulation of blast/promyelocytic cells in the bone marrow and spleen in the absence of leukocytosis resembling acute promyelocytic leukemia
• leukemia is also characterized by infiltrating cells that are c-Kit, Gr-1, Mac-1 positive and display promyelocytic features

Mouse Models of Human Disease
OMIM ID Ref(s)
Acute Promyelocytic Leukemia; APL 612376 J:65878




Genotype
MGI:5514339
cx2
Allelic
Composition
Tg(CTSG-RARA/ZBTB16)#Ppp/0
Tg(CTSG-ZBTB16/RARA)#Ppp/0
Genetic
Background
Not Specified
Find Mice Using the International Mouse Strain Resource (IMSR)
No mouse lines available in IMSR.
See publication links below for author information.
phenotype observed in females
phenotype observed in males
N normal phenotype
cellular
• overall rate of bone marrow cell apoptosis and growth factor deprivation-induced programmed cell death is reduced

hematopoietic system
• clonogenic capacity of hematopoietic precursors is reduced, with a reduced number of colony-forming unit-granulocyte, macrophage (CFU-GM)
• leukemia is preceded by a myeloproliferative disorder characterized by the accumulation of immature myeloid cells and promyelocytes in the bone marrow and spleen, indicating impaired myeloid differentiation
• low platelet counts in the peripheral blood
• mild increase in white blood cell counts in the peripheral blood
• leukocytosis is much less pronounced than in single Tg(CTSG-ZBTB16/RARA)#Ppp mutants
• proliferation rate of bone marrow cells is higher
• the number of leukemic clusters (small colonies of 8-20 cells) observed in bone marrow is increased compared to wild-type bone marrow
• overall rate of bone marrow cell apoptosis and growth factor deprivation-induced programmed cell death is reduced

immune system
• leukemia is preceded by a myeloproliferative disorder characterized by the accumulation of immature myeloid cells and promyelocytes in the bone marrow and spleen, indicating impaired myeloid differentiation
• mild increase in white blood cell counts in the peripheral blood
• leukocytosis is much less pronounced than in single Tg(CTSG-ZBTB16/RARA)#Ppp mutants

neoplasm
• 100% penetrance of leukemia after 6 months of age
• leukemias are unresponsive to retinoic acid and survival following treatment is shorter (30 days) than in untreated mutants (4 weeks from diagnosis)
• retinoic acid-induced differentiation in leukemic cells is reduced compared to single single Tg(CTSG-ZBTB16/RARA)#Ppp mutants
• leukemia is characterized by an accumulation of immature blasts and of cells blocked at the promyelocytic stage of differentiation (more than 50% of promyelocytes in bone marrow in some cases)
• leukemia is also characterized by a significant accumulation of c-Kit+/CD34+ and Gr-1+/Mac-1- cells representing early myeloid progenitors in the bone marrow and spleen

Mouse Models of Human Disease
OMIM ID Ref(s)
Acute Promyelocytic Leukemia; APL 612376 J:65878




Genotype
MGI:5514273
tg3
Allelic
Composition
Tg(CTSG-ZBTB16/RARA)#Ppp/0
Genetic
Background
Not Specified
Find Mice Using the International Mouse Strain Resource (IMSR)
No mouse lines available in IMSR.
See publication links below for author information.
phenotype observed in females
phenotype observed in males
N normal phenotype
hematopoietic system
• leukemia is preceded by a myeloproliferative disorder of variable duration characterized by progressive accumulation of myeloid precursors in the bone marrow and spleen, and in some cases the peripheral blood

immune system
• leukemia is preceded by a myeloproliferative disorder of variable duration characterized by progressive accumulation of myeloid precursors in the bone marrow and spleen, and in some cases the peripheral blood

mortality/aging
• all mice die of leukemia between 6 months and 1.5 years of age

neoplasm
• all mice develop leukemia
• leukemia is resistant to retinoic acid treatment and complete remission is not observed, although survival is prolonged and hematopoietic differentiation is induced
• however, much higher doses than normal of retinoic acid treatment leads to remission
• leukemia is characterized by leukocytosis in peripheral blood and infiltration in all organs by myeloid leukemic progenitors with promyelocityic and myelocytic features that retain the capacity to terminally mature, resembling chronic myeloid leukemia (J:78201)





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last database update
07/19/2016
MGI 6.04
The Jackson Laboratory