mortality/aging
• mice die between 3 and 4 weeks
|
immune system
• osteoclast differentiation is halted prior to TRAP expression
|
• B cell developmental block occurs at the immature IgM stage
• the block in B cell development is maintained when cells are adoptively transferred into in Rag1 null mice
|
• mice exhibit a higher proportional of granulocytes in the spleen compared to in wild-type mice due to a decrease in other cell types
• however, bone marrow granulocyte numbers are normal
|
• fewer M342+ interdigitating dendritic cells are present and are disorganized compared to in wild-type mice
• thymic medullary dendritic cells are reduced in number and disorganized compared to in wild-type mice
|
• B220+ B cells in the spleen are decreased 1.3- to 2.6-fold compared to in wild-type mice
• IgM+ B cells are reduced and IgD+ cells are undetected unlike in wild-type mice
|
• no T cells are detected in the spleen
|
• reduction in dull staining double positive T cells
|
• single positive T cells are low in the thymus and undetected in the periphery unlike in wild-type mice
• however, single T cells are produced when transferred into Rag1 null mice
|
• no TRAP+ osteoclasts are detected unlike in wild-type mice
• however, no abnormal apoptosis of osteoclasts is detected
|
• mice exhibit a higher proportional of macrophages in the spleen compared to in wild-type mice due to a decrease in other cell types
• however, bone marrow macrophage numbers are normal
|
• mature macrophage functions are impaired
|
• thymic cortical epithelial cells are randomly distributed unlike in wild-type mice
|
• medullary epithelial cells are absent
|
• 10- to 30-fold fewer cells
|
• B220+ B cells are diffusely distributed throughout the spleen with only a few rare T cells and macrophages present throughout the spleen unlike in wild-type mice
|
skeleton
• osteoclast differentiation is halted prior to TRAP expression
|
• incisors fail to erupt
• however, adoptive transfer with wild-type liver cells restores tooth eruption by 5 weeks
|
• no TRAP+ osteoclasts are detected unlike in wild-type mice
• however, no abnormal apoptosis of osteoclasts is detected
|
• long bones are shortened, radio-opaque, and lack proper bone marrow cavities unlike in wild-type mice
|
• long bones lack proper bone marrow cavities
|
osteopetrosis
(
J:45119
)
• at 2 to 4 weeks, mice develop osteopetrosis
• however, adoptive transfer with wild-type bone marrow cells rescues osteopetrosis
|
growth/size/body
• incisors fail to erupt
• however, adoptive transfer with wild-type liver cells restores tooth eruption by 5 weeks
|
• within a week of birth
|
craniofacial
• incisors fail to erupt
• however, adoptive transfer with wild-type liver cells restores tooth eruption by 5 weeks
|
hematopoietic system
• osteoclast differentiation is halted prior to TRAP expression
|
• thymic cortical epithelial cells are randomly distributed unlike in wild-type mice
|
• medullary epithelial cells are absent
|
• 10- to 30-fold fewer cells
|
• B cell developmental block occurs at the immature IgM stage
• the block in B cell development is maintained when cells are adoptively transferred into in Rag1 null mice
|
• mice exhibit a higher proportional of granulocytes in the spleen compared to in wild-type mice due to a decrease in other cell types
• however, bone marrow granulocyte numbers are normal
|
• fewer M342+ interdigitating dendritic cells are present and are disorganized compared to in wild-type mice
• thymic medullary dendritic cells are reduced in number and disorganized compared to in wild-type mice
|
• B220+ B cells in the spleen are decreased 1.3- to 2.6-fold compared to in wild-type mice
• IgM+ B cells are reduced and IgD+ cells are undetected unlike in wild-type mice
|
• no T cells are detected in the spleen
|
• reduction in dull staining double positive T cells
|
• single positive T cells are low in the thymus and undetected in the periphery unlike in wild-type mice
• however, single T cells are produced when transferred into Rag1 null mice
|
• no TRAP+ osteoclasts are detected unlike in wild-type mice
• however, no abnormal apoptosis of osteoclasts is detected
|
• mice exhibit a higher proportional of macrophages in the spleen compared to in wild-type mice due to a decrease in other cell types
• however, bone marrow macrophage numbers are normal
|
• B220+ B cells are diffusely distributed throughout the spleen with only a few rare T cells and macrophages present throughout the spleen unlike in wild-type mice
|
• mature macrophage functions are impaired
|
cellular
• osteoclast differentiation is halted prior to TRAP expression
|
endocrine/exocrine glands
• thymic cortical epithelial cells are randomly distributed unlike in wild-type mice
|
• medullary epithelial cells are absent
|
• 10- to 30-fold fewer cells
|