Phenotypes Associated with This Genotype

Genotype
MGI:3580416

cx68

• Allelic Composition: Rag1^tm1Mom/Rag1^tm1Mom; Prf1^tm1Sdz/Prf1^tm1Sdz
• Genetic Background: NOD.Cg-Rag1^tm1Mom Prf1^tm1Sdz/Sz

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

mortality/aging

premature death ( J:109843 )

• mean lifespan is 262+/-21 days

immune system

abnormal thymus cortex morphology ( J:109843 )

• thymus lacks a clearly defined cortex

thymus hypoplasia ( J:109843 )

• lymphoid cells are severely reduced in thymuses

increased T cell derived lymphoma incidence ( J:109843 )

increased immature B cell number ( J:109843 )

• IgLkappa- B220+ immature B cells are increased six-fold as compared to NOD controls

increased granulocyte number ( J:109843 )

• numbers of granulocytes are increased in comparison to NOD controls, however, in comparison to NOD-Rag^null numbers are decreased

decreased mature B cell number ( J:109843 )

• spleens of 8-11 week old mice are deficient in Igkappa+ B220+ T cells

increased NK cell number ( J:109843 )

• numbers of NK cells are increased in comparison to NOD controls

decreased CD4-positive, alpha-beta T cell number ( J:109843 )

• spleens of 8-11 week old mice are deficient in CD3+ CD4+ T cells

decreased CD8-positive, alpha-beta T cell number ( J:109843 )

• spleens of 8-11 week old mice are deficient in CD3+ CD8+ T cells

increased macrophage cell number ( J:109843 )

• numbers of macrophages are increased in comparison to NOD controls

spleen hypoplasia ( J:109843 )

• nucleated spleen cells are reduced 14 fold in 8-11 week old mice as compared to NOD control

abnormal spleen white pulp morphology ( J:109843 )

• spleen lacks lymphoid follicles

decreased immunoglobulin level ( J:109843 )

• no serum immunoglobulin is detected in 176-362 day old mice

impaired natural killer cell mediated cytotoxicity ( J:109843 )

• poly I:C stimulated spleen cells exhibit extremely low levels of NK cell cyotoxic activity

abnormal lymph node cortex morphology ( J:109843 )

• lymph nodes lack follicles

abnormal level of surface class II molecules ( J:109843 )

• four to five-fold decrease in cells expressing I-A^g7 as compared to NOD control

abnormal response to transplant ( J:109843 )

• following IP injection of human PMBC, peripheral blood has a fivefold increase in the percentage of human lymphoid cells as compared to NOD-Rag^null controls

• peripheral blood has a 13-fold increase in engraftment of human CD4+ T cells

• spleens have a 9-fold increase in engraftment of human CD45+ cells and a 20-fold increase in human CD4+ cells as compared to control, although there is no significant difference in engraftment of human CD8+ cells

• 6-8 weeks after IP injection, bone marrow exhibits a 12-fold increase in engraftment of human cord blood cells

neoplasm

increased T cell derived lymphoma incidence ( J:109843 )

hematopoietic system

abnormal thymus cortex morphology ( J:109843 )

• thymus lacks a clearly defined cortex

thymus hypoplasia ( J:109843 )

• lymphoid cells are severely reduced in thymuses

increased T cell derived lymphoma incidence ( J:109843 )

increased immature B cell number ( J:109843 )

• IgLkappa- B220+ immature B cells are increased six-fold as compared to NOD controls

increased granulocyte number ( J:109843 )

• numbers of granulocytes are increased in comparison to NOD controls, however, in comparison to NOD-Rag^null numbers are decreased

decreased mature B cell number ( J:109843 )

• spleens of 8-11 week old mice are deficient in Igkappa+ B220+ T cells

increased NK cell number ( J:109843 )

• numbers of NK cells are increased in comparison to NOD controls

decreased CD4-positive, alpha-beta T cell number ( J:109843 )

• spleens of 8-11 week old mice are deficient in CD3+ CD4+ T cells

decreased CD8-positive, alpha-beta T cell number ( J:109843 )

• spleens of 8-11 week old mice are deficient in CD3+ CD8+ T cells

increased macrophage cell number ( J:109843 )

• numbers of macrophages are increased in comparison to NOD controls

spleen hypoplasia ( J:109843 )

• nucleated spleen cells are reduced 14 fold in 8-11 week old mice as compared to NOD control

abnormal spleen white pulp morphology ( J:109843 )

• spleen lacks lymphoid follicles

decreased immunoglobulin level ( J:109843 )

• no serum immunoglobulin is detected in 176-362 day old mice

impaired natural killer cell mediated cytotoxicity ( J:109843 )

• poly I:C stimulated spleen cells exhibit extremely low levels of NK cell cyotoxic activity

endocrine/exocrine glands

abnormal thymus cortex morphology ( J:109843 )

• thymus lacks a clearly defined cortex

thymus hypoplasia ( J:109843 )

• lymphoid cells are severely reduced in thymuses

increased T cell derived lymphoma incidence ( J:109843 )