hematopoietic system
• mice develop a variable and progressive splenomegaly that is more severe than in heterozygous mice
|
• bone marrow cells exhibit a two-fold increase in the Lin-Sca1+cKit+ (LSK) compartment compared to in wild-type and heterozygous mice
• monocytes are present in the bone marrow and liver in a background of increased number of maturing myeloid cells is increased compared to in wild-type mice
• the relative percentage of immunophenotypic granulocyte-monocyte progenitors is expanded while the percent of megakaryocyte-erythroid progenitors is decreased
• however, the total number of myeloid progenitor cells is the same as in wild-type mice
• the proportion of LSK cells in S/G2/M phase is increased while the proportion in G0/G1 is increased compared to in wild-type mice
• fewer LSK cells undergo apoptosis compared to in wild-type and heterozygous mice
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• when cultured on methylcellulose, bone marrow cells produce more colonies than wild-type cells with a greater number of monocytes and fewer erythroid burst forming units and granulocyte-erythroid burst forming units compared to wild-type and heterozygous mice
• however, re-plated colonies do not exhibit increased self-renewal properties
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• B cell maturation is blocked at the pre-B stage
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• the rise in monocytes in the bone marrow and spleen is accompanied by a decrease in the percentage of T and B lymphoid cells
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• the number of mature B cells in the bone marrow and spleen is decreased compared to in wild-type mice
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• the rise in monocytes in the bone marrow and spleen is accompanied by a decrease in the percentage of T and B lymphoid cells
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• mice exhibit leukocytosis and monocytosis with a reversal in the neutrophil to lymphocyte ratio compared to in wild-type mice
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• granulocyte populations are increased in peripheral blood compared to in wild-type mice
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• monocyte populations are increased in peripheral blood
• monocytes are present in the bone marrow and liver in a background of increased number of maturing myeloid cells is increased compared to in wild-type mice
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• expanded red pulp is comprised of mainly mature myeloid and ad-mixed erythroid elements
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• expanded white pulp is comprised of cells resembling maturing monocytes
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immune system
• mice develop a variable and progressive splenomegaly that is more severe than in heterozygous mice
|
• B cell maturation is blocked at the pre-B stage
|
• the rise in monocytes in the bone marrow and spleen is accompanied by a decrease in the percentage of T and B lymphoid cells
|
• the number of mature B cells in the bone marrow and spleen is decreased compared to in wild-type mice
|
• the rise in monocytes in the bone marrow and spleen is accompanied by a decrease in the percentage of T and B lymphoid cells
|
• mice exhibit leukocytosis and monocytosis with a reversal in the neutrophil to lymphocyte ratio compared to in wild-type mice
|
• granulocyte populations are increased in peripheral blood compared to in wild-type mice
|
• monocyte populations are increased in peripheral blood
• monocytes are present in the bone marrow and liver in a background of increased number of maturing myeloid cells is increased compared to in wild-type mice
|
• expanded red pulp is comprised of mainly mature myeloid and ad-mixed erythroid elements
|
• expanded white pulp is comprised of cells resembling maturing monocytes
|
growth/size/body
• mice develop a variable and progressive splenomegaly that is more severe than in heterozygous mice
|
Mouse Models of Human Disease |
DO ID | OMIM ID(s) | Ref(s) | |
juvenile myelomonocytic leukemia | DOID:0050458 |
OMIM:607785 |
J:126003 |