Analysis Tools|
Allele Symbol Allele Name Allele ID |
Ctsgtm2.1(PML/RARA)Ley targeted mutation 2.1, Timothy J Ley MGI:3526413 |
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| Summary |
5 genotypes
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| ♀ | phenotype observed in females |
| ♂ | phenotype observed in males |
| N | normal phenotype |
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• homozygotes develop an abrupt onset, rapidly fatal acute leukemia with a median age of onset of 6 months and penetrance of over 90%
• median age of onset is earlier than in heterozygotes, however the leukemia phenotype and penetrance are the same as in heterozygous mutants
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| ♀ | phenotype observed in females |
| ♂ | phenotype observed in males |
| N | normal phenotype |
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• median survival is 473 days
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• bone marrow cells exhibit increased replating efficiency as determined by increased colony formation compared with wild-type bone marrow
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• mice exhibit an increase in Gr-1+ and Gr-1+/CD117+ cells in the spleen compared with wild-type mice
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• mice exhibit an increase in Gr-1+ and Gr-1+/CD117+ cells in the spleen compared with wild-type mice
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• most mice develop acute myeloid leukemia with promyelocytic features, including periperal leukocytosis and infiltration of the spleen and bone marrow with Gr-1+ CD117+ tumor cells unlike wild-type mice
• median tumor free survival is increased compared to in Ctsgtm3.1(PML/RARA)Ley heterozygotes
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• mice exhibit an increase in Gr-1+ and Gr-1+/CD117+ cells in the spleen compared with wild-type mice
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| ♀ | phenotype observed in females |
| ♂ | phenotype observed in males |
| N | normal phenotype |
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• heterozygotes develop an abrupt onset, rapidly fatal acute leukemia with a median age of overt onset of 10 months and penetrance of over 90%
(J:84230)
• heterozygotes develop acute promyelocytic leukemia with a 150-200 day latency and full penetrance by 350 days of age
(J:86458)
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• progressive splenomegaly is seen prior to the onset of leukemia
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• increased numbers of myeloblasts and promyelocytes are seen in mutants with leukemia
(J:84230)
• splenic myeloid expansion and aberrant CD34 Gr-1 double positive cells are seen in all mutants
(J:86458)
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• extramedullary hematopoiesis is seen prior to the onset of leukemia
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• seen in mutants with leukemia
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• pronounced leukocytosis is seen in mutants with leukemia
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• progressive splenomegaly is seen prior to the onset of leukemia
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• increased numbers of myeloblasts and promyelocytes are seen in mutants with leukemia
(J:84230)
• splenic myeloid expansion and aberrant CD34 Gr-1 double positive cells are seen in all mutants
(J:86458)
|
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• pronounced leukocytosis is seen in mutants with leukemia
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• hepatomegaly with leukemic cell infiltrates is seen in mutants with leukemia
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• hepatomegaly with leukemic cell infiltrates is seen in mutants with leukemia
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• progressive splenomegaly is seen prior to the onset of leukemia
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Mouse Models of Human Disease |
DO ID | OMIM ID(s) | Ref(s) | |
| acute myeloid leukemia | DOID:9119 |
OMIM:601626 |
J:84230 , J:86458 | |
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| ♀ | phenotype observed in females |
| ♂ | phenotype observed in males |
| N | normal phenotype |
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• compound heterozygotes develop an abrupt onset, rapidly fatal acute leukemia with age of onset and penetrance similar to Ctsgtm2.1Ley heterozygotes
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| ♀ | phenotype observed in females |
| ♂ | phenotype observed in males |
| N | normal phenotype |
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• heterozygotes develop acute promyelocytic leukemia with only 45% penetrance by 350 days of age, despite the decreased incidence of leukemia the phenotype of the leukemias was the same as in Ctsgtm2.1Ley heterozygotes
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• splenic myeloid expansion and aberrant CD34 Gr-1 double positive cells are seen in all mutants
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• splenic myeloid expansion and aberrant CD34 Gr-1 double positive cells are seen in all mutants
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Mouse Genome Database (MGD), Gene Expression Database (GXD), Mouse Models of Human Cancer database (MMHCdb) (formerly Mouse Tumor Biology (MTB)), Gene Ontology (GO) |
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last database update 12/10/2024 MGI 6.24 |
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