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Phenotypes Associated with This Genotype
Genotype
MGI:3845014
Allelic
Composition
Ptpn11tm6Bgn/Ptpn11+
Tg(Mx1-cre)1Cgn/0
Genetic
Background
involves: 129S6/SvEvTac * C57BL/6 * CBA
Find Mice Using the International Mouse Strain Resource (IMSR)
Mouse lines carrying:
Ptpn11tm6Bgn mutation (2 available); any Ptpn11 mutation (11 available)
Tg(Mx1-cre)1Cgn mutation (6 available)
phenotype observed in females
phenotype observed in males
N normal phenotype
mortality/aging
• all mice die within 45 weeks after receiving pIpC injections

hematopoietic system
• following pIpC injections cultured bone marrow megakaryocyte-erythrocyte progenitor cells produce fewer erythrocyte colony-forming unit colonies and more, larger erythroid burst-forming unit colonies
• the myeloproliferative disorder seen after pIpC injections does not develop in irradiated mice engrafted with cells from diseased mice
• following pIpC injections common myeloid progenitor cell numbers are reduced in the bone marrow
• following pIpC injections cultured erythroid progenitors produce fewer erythrocyte colony-forming unit colonies
• following pIpC injections both bone marrow and spleen cells form increased numbers of cytokine independent colonies that are primarily macrophage colony forming units and granulocyte colony forming units
• following pIpC injections mice develop marked extramedullary hematopoiesis with an increase in the numbers of long term and short term hematopoietic stem cells and granulocyte-monocyte progenitor cells following pIpC injections
• following pIpC injections mice develop anemia
• following pIpC injections increased numbers of immature predominantly granulocytic cells are found in the bone marrow
• following pIpC injections total bone marrow cellularity is decreased
• following pIpC injections granulocyte-monocyte progenitor cell numbers are reduced in the bone marrow
• following pIpC injections mice develop progressive leukocytosis
• following pIpC injections mice develop progressive granulocytosis
• following pIpC injections mice develop progressive monocytosis
• following pIpC injections the sizes of the Lin-Sca1+cKit+ (LSK) and Lin-Sca1-cKit+ (LK) compartments in the bone marrow are reduced
• following pIpC injections fewer cells in the LSK compartment are quiescent and these cells are hypersensitive to stem cell factor
• following pIpC injections the number of long term hematopoietic stem cells is reduced
• following pIpC injections infiltration of mature myeloid cells into the red pulp is seen
• following pIpC injections mice develop splenomegaly
• following pIpC injections the ratio of Mac1+Gr1+ cells is increased 9 to 10 fold, the ratio of erythroid progenitors is increased 6 to 7 fold, and the relative number of T cells is decreased

liver/biliary system
• following pIpC injections periportal cuffing of liver sinusoids with infiltrating granulocytes is seen
• following pIpC injections mice develop hepatomegaly

immune system
• following pIpC injections both bone marrow and spleen cells form increased numbers of cytokine independent colonies that are primarily macrophage colony forming units and granulocyte colony forming units
• following pIpC injections mice develop progressive leukocytosis
• following pIpC injections mice develop progressive granulocytosis
• following pIpC injections mice develop progressive monocytosis
• following pIpC injections infiltration of mature myeloid cells into the red pulp is seen
• following pIpC injections mice develop splenomegaly
• following pIpC injections the ratio of Mac1+Gr1+ cells is increased 9 to 10 fold, the ratio of erythroid progenitors is increased 6 to 7 fold, and the relative number of T cells is decreased

cardiovascular system
• following pIpC injections periportal cuffing of liver sinusoids with infiltrating granulocytes is seen

Mouse Models of Human Disease
DO ID OMIM ID(s) Ref(s)
juvenile myelomonocytic leukemia DOID:0050458 OMIM:607785
J:148430


Contributing Projects:
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
02/18/2020
MGI 6.14
The Jackson Laboratory