Phenotypes Associated with This Genotype

Genotype
MGI:5009037

• Allelic Composition: Ncstn^tm1.1Akli/Ncstn^tm1.1Akli; Tg(VAV1-cre)1Graf/0
• Genetic Background: involves: 129 * C57BL/6 * SJL

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

growth/size/body

enlarged spleen ( J:172442 )

mortality/aging

premature death ( J:172442 )

• do not survive more than 20 weeks

hematopoietic system

abnormal definitive hematopoiesis ( J:172442 )

• significant reduction of the lymphoid-biased multipotential progenitor population (L-MPP)

• striking increase in the absolute numbers of both bone marrow and spleen granulocyte/monocyte progenitors cells

• decrease of the megakaryocyte?erythrocyte progenitor population

• progenitor cells display an increase in their self-renewal capacity

increased leukocyte cell number ( J:172442 )

• striking peripheral blood leukocytosis

increased monocyte cell number ( J:172442 )

• striking peripheral blood monocytosis

• increase in monocyte numbers in the bone marrow and liver

abnormal hematopoietic stem cell morphology ( J:172442 )

• de-repression of an extended myeloid-specific program in LSK cells

increased hematopoietic stem cell number ( J:172442 )

• increase in LSK numbers

abnormal spleen morphology ( J:172442 )

• expansion of the red pulp with diffuse infiltration by myeloid and monocytic cells

enlarged spleen ( J:172442 )

increased spleen red pulp amount ( J:172442 )

• expansion of the red pulp

neoplasm

increased leukemia incidence ( J:172442 )

• similarity to chronic myelomonocytic leukemia

immune system

increased leukocyte cell number ( J:172442 )

• striking peripheral blood leukocytosis

increased monocyte cell number ( J:172442 )

• striking peripheral blood monocytosis

• increase in monocyte numbers in the bone marrow and liver

abnormal spleen morphology ( J:172442 )

• expansion of the red pulp with diffuse infiltration by myeloid and monocytic cells

enlarged spleen ( J:172442 )

increased spleen red pulp amount ( J:172442 )

• expansion of the red pulp

Mouse Models of Human Disease		DO ID	OMIM ID(s)	Ref(s)
chronic myeloid leukemia		DOID:8552	OMIM:608232	J:172442