All Phenotypes Ctsg<tm2.1(PML/RARA)Ley> MGI Mouse

Phenotypes associated with this allele

Jump to	Allelic Composition	Genetic Background	Genotype ID
hm1	Ctsg^{tm2.1(PML/RARA)Ley}/Ctsg^{tm2.1(PML/RARA)Ley}	involves: 129X1/SvJ * C57BL/6	MGI:3526659
ht2	Ctsg^{tm2.1(PML/RARA)Ley}/Ctsg⁺	involves: 129 * 129X1/SvJ * C57BL/6	MGI:4847634
ht3	Ctsg^{tm2.1(PML/RARA)Ley}/Ctsg⁺	involves: 129X1/SvJ * C57BL/6	MGI:3526657
ht4	Ctsg^tm1Ley/Ctsg^{tm2.1(PML/RARA)Ley}	involves: 129X1/SvJ * C57BL/6	MGI:3526661
cx5	Ctsg^{tm2.1(PML/RARA)Ley}/Ctsg⁺ Elane^tm1Sds/Elane^tm1Sds	involves: 129S/SvEv * 129X1/SvJ * C57BL/6	MGI:3526663

Allelic Composition	Ctsg^{tm2.1(PML/RARA)Ley}/Ctsg^{tm2.1(PML/RARA)Ley}
Genetic Background	involves: 129X1/SvJ * C57BL/6

Find Mice

Using the International Mouse Strain Resource (IMSR)
Mouse lines carrying:
Ctsg^{tm2.1(PML/RARA)Ley} mutation (0 available); any Ctsg mutation (13 available)

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

neoplasm

increased leukemia incidence ( J:84230 )

• 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

Allelic Composition	Ctsg^{tm2.1(PML/RARA)Ley}/Ctsg⁺
Genetic Background	involves: 129 * 129X1/SvJ * C57BL/6

Find Mice

Using the International Mouse Strain Resource (IMSR)
Mouse lines carrying:
Ctsg^{tm2.1(PML/RARA)Ley} mutation (0 available); any Ctsg mutation (13 available)

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

mortality/aging

premature death ( J:166479 )

• median survival is 473 days

hematopoietic system

abnormal bone marrow cell morphology/development ( J:166479 )

• bone marrow cells exhibit increased replating efficiency as determined by increased colony formation compared with wild-type bone marrow

abnormal common myeloid progenitor cell morphology ( J:166479 )

• mice exhibit an increase in Gr-1+ and Gr-1+/CD117+ cells in the spleen compared with wild-type mice

abnormal spleen morphology ( J:166479 )

• mice exhibit an increase in Gr-1+ and Gr-1+/CD117+ cells in the spleen compared with wild-type mice

enlarged spleen ( J:166479 )

increased spleen weight ( J:166479 )

neoplasm

increased acute promyelocytic leukemia incidence ( J:166479 )

• 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 Ctsg^{tm3.1(PML/RARA)Ley} heterozygotes

immune system

abnormal spleen morphology ( J:166479 )

• mice exhibit an increase in Gr-1+ and Gr-1+/CD117+ cells in the spleen compared with wild-type mice

enlarged spleen ( J:166479 )

increased spleen weight ( J:166479 )

growth/size/body

enlarged spleen ( J:166479 )

increased spleen weight ( J:166479 )

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

neoplasm

increased leukemia incidence ( J:84230 , J:86458 )

• 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)

hematopoietic system

enlarged spleen ( J:84230 )

• progressive splenomegaly is seen prior to the onset of leukemia

abnormal myelopoiesis ( J:84230 , J:86458 )

• 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)

extramedullary hematopoiesis ( J:84230 )

• extramedullary hematopoiesis is seen prior to the onset of leukemia

anemia ( J:84230 )

• seen in mutants with leukemia

thrombocytopenia ( J:84230 )

• seen in mutants with leukemia

increased leukocyte cell number ( J:84230 )

• pronounced leukocytosis is seen in mutants with leukemia

immune system

enlarged spleen ( J:84230 )

• progressive splenomegaly is seen prior to the onset of leukemia

abnormal myelopoiesis ( J:84230 , J:86458 )

• 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)

increased leukocyte cell number ( J:84230 )

• pronounced leukocytosis is seen in mutants with leukemia

liver/biliary system

enlarged liver ( J:84230 )

• hepatomegaly with leukemic cell infiltrates is seen in mutants with leukemia

growth/size/body

enlarged liver ( J:84230 )

• hepatomegaly with leukemic cell infiltrates is seen in mutants with leukemia

enlarged spleen ( J:84230 )

• progressive splenomegaly is seen prior to the onset of leukemia

Mouse Models of Human Disease		DO ID	OMIM ID(s)	Ref(s)
acute myeloid leukemia		DOID:9119	OMIM:601626	J:84230 , J:86458

Allelic Composition	Ctsg^tm1Ley/Ctsg^{tm2.1(PML/RARA)Ley}
Genetic Background	involves: 129X1/SvJ * C57BL/6

Find Mice

Using the International Mouse Strain Resource (IMSR)
Mouse lines carrying:
Ctsg^tm1Ley mutation (0 available); any Ctsg mutation (13 available)
Ctsg^{tm2.1(PML/RARA)Ley} mutation (0 available); any Ctsg mutation (13 available)

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

neoplasm

increased leukemia incidence ( J:84230 )

• compound heterozygotes develop an abrupt onset, rapidly fatal acute leukemia with age of onset and penetrance similar to Ctsg^tm2.1Ley heterozygotes

Allelic Composition	Ctsg^{tm2.1(PML/RARA)Ley}/Ctsg⁺ Elane^tm1Sds/Elane^tm1Sds
Genetic Background	involves: 129S/SvEv * 129X1/SvJ * C57BL/6

Find Mice

Using the International Mouse Strain Resource (IMSR)
Mouse lines carrying:
Ctsg^{tm2.1(PML/RARA)Ley} mutation (0 available); any Ctsg mutation (13 available)
Elane^tm1Sds mutation (2 available); any Elane mutation (21 available)

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

neoplasm

increased leukemia incidence ( J:86458 )

• 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 Ctsg^tm2.1Ley heterozygotes

hematopoietic system

abnormal myelopoiesis ( J:86458 )

• splenic myeloid expansion and aberrant CD34 Gr-1 double positive cells are seen in all mutants

immune system

abnormal myelopoiesis ( J:86458 )

• splenic myeloid expansion and aberrant CD34 Gr-1 double positive cells are seen in all mutants

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