Phenotypes Associated with This Genotype

Genotype
MGI:4360783

• Allelic Composition: Ikzf1^tm1Kge/Ikzf1⁺
• Genetic Background: involves: 129S4/SvJae * C57BL/6

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

mortality/aging

premature death ( J:29355 )

• mice develop a fatal leukemia between 3 and 6 months of age

neoplasm

increased leukemia incidence ( J:29355 )

• leukemia derived from alpha-beta T cells occurs in all mice

• the cells are large, can be either CD4⁺ or CD8⁺, and are monoclonal in their use of TCR beta chains

• these lymphoblastic cells accumulate in all immune organs and in the liver, kidney, and lung

• leukemia cells disrupt the architecture of numerous organs as the mice age

• studies with 2-month old mice suggest the malignant cells arise in the thymus and not in the spleen

immune system

increased T cell proliferation ( J:29355 )

• basal proliferation of T cells is 2.6 fold higher than controls

abnormal thymus lobule morphology ( J:29355 )

• leukemia leads to the loss of the bi-lobed structure of the thymus in mice over 3 months of age

thymus cortex hypoplasia ( J:29355 )

• leukemia in older mice leads to complete effacement of cortical and medullary structures in the thymus

abnormal thymus medulla morphology ( J:29355 )

• leukemia in older mice leads to complete effacement of cortical and medullary structures in the thymus

enlarged thymus ( J:29355 )

• leukemia leads to a swelling of the thymus in mice over 3 months of age

abnormal thymocyte activation ( J:29355 )

• thymocytes from 1 month old mice have a 200-fold increase in their proliferation rate in response to TCR stimulus compared to controls

• a less dramatic increase in proliferation rate is observed with thymocytes from E18.5 fetuses

spleen hyperplasia ( J:29355 )

• splenocyte numbers are 2- to 5- fold greater than controls by 3 months of age

• leukemia later in life leads to spleen cellularity that is 10-fold greater than normal

abnormal T cell differentiation ( J:29355 )

• starting at ages 2-3 months, intermediate single positive thymoyctes start accumulating

decreased spleen red pulp amount ( J:29355 )

• leukemia leads to an decrease in the red pulp area of the spleen

increased spleen white pulp amount ( J:29355 )

• leukemia leads to an increase in the white pulp area of the spleen

enlarged mesenteric lymph nodes ( J:29355 )

• leukemia results in enlarged lymph nodes for all mice over 3 months of age

• lymph nodes enlarge 20- to 50- fold greater than normal

abnormal lymph node cortex morphology ( J:29355 )

• leukemia leads to the loss of lymph node structure

abnormal lymph node medulla morphology ( J:29355 )

• leukemia leads to the loss of lymph node structure

lymph node hyperplasia ( J:29355 )

• leukemia results in enlarged lymph nodes for all mice over 3 months of age

• lymph nodes enlarge 20- to 50- fold greater than normal

enlarged cervical lymph nodes ( J:29355 )

• leukemia results in enlarged lymph nodes for all mice over 3 months of age

• lymph nodes enlarge 20- to 50- fold greater than normal

hematopoietic system

increased T cell proliferation ( J:29355 )

• basal proliferation of T cells is 2.6 fold higher than controls

abnormal thymus lobule morphology ( J:29355 )

• leukemia leads to the loss of the bi-lobed structure of the thymus in mice over 3 months of age

thymus cortex hypoplasia ( J:29355 )

• leukemia in older mice leads to complete effacement of cortical and medullary structures in the thymus

abnormal thymus medulla morphology ( J:29355 )

• leukemia in older mice leads to complete effacement of cortical and medullary structures in the thymus

enlarged thymus ( J:29355 )

• leukemia leads to a swelling of the thymus in mice over 3 months of age

abnormal thymocyte activation ( J:29355 )

• thymocytes from 1 month old mice have a 200-fold increase in their proliferation rate in response to TCR stimulus compared to controls

• a less dramatic increase in proliferation rate is observed with thymocytes from E18.5 fetuses

spleen hyperplasia ( J:29355 )

• splenocyte numbers are 2- to 5- fold greater than controls by 3 months of age

• leukemia later in life leads to spleen cellularity that is 10-fold greater than normal

abnormal T cell differentiation ( J:29355 )

• starting at ages 2-3 months, intermediate single positive thymoyctes start accumulating

decreased spleen red pulp amount ( J:29355 )

• leukemia leads to an decrease in the red pulp area of the spleen

increased spleen white pulp amount ( J:29355 )

• leukemia leads to an increase in the white pulp area of the spleen

endocrine/exocrine glands

abnormal thymus lobule morphology ( J:29355 )

• leukemia leads to the loss of the bi-lobed structure of the thymus in mice over 3 months of age

thymus cortex hypoplasia ( J:29355 )

• leukemia in older mice leads to complete effacement of cortical and medullary structures in the thymus

abnormal thymus medulla morphology ( J:29355 )

• leukemia in older mice leads to complete effacement of cortical and medullary structures in the thymus

enlarged thymus ( J:29355 )

• leukemia leads to a swelling of the thymus in mice over 3 months of age

abnormal thymocyte activation ( J:29355 )

• thymocytes from 1 month old mice have a 200-fold increase in their proliferation rate in response to TCR stimulus compared to controls

• a less dramatic increase in proliferation rate is observed with thymocytes from E18.5 fetuses

cellular

increased T cell proliferation ( J:29355 )

• basal proliferation of T cells is 2.6 fold higher than controls

growth/size/body

spleen hyperplasia ( J:29355 )

• splenocyte numbers are 2- to 5- fold greater than controls by 3 months of age

• leukemia later in life leads to spleen cellularity that is 10-fold greater than normal