Analysis Tools
mortality/aging
|
• animals die between 3-20 weeks of age from repeated illnesses
|
|
• about 50% of animals die between postnatal days 1-3; pups are selectively cannibalized by their mothers
|
behavior/neurological
|
• ataxic gait is first noticeable at 2-4 weeks of age and becomes progressively more severe with age
• walking is uncoordinated and mice fall over onto their side frequently
|
growth/size/body
|
• mice that survive the first few days after birth are smaller
|
hematopoietic system
|
• proliferative response of adult, but not newborn, splenocytes to LPS or ConA stimulation is reduced 8-10-fold compared to wild-type
|
|
• number of thymocytes is decreased to less than 1% of wild-type
|
|
• involuted thymus
|
|
• hematopoieisis is severely reduced
• hematopoietic cell counts are reduced to about 30% of wild-type levels
|
|
• absolute number of immature B cells (B220+HSA+ and B220+BPl+) is significantly reduced
|
|
• the immature CD4-CD8- thymocyte population is increased from 4% of total cells in wild-type to 90% of total cells in mutants, although in absolute numbers, this population size is decreased to 12% of wild-type, indicating the presence of mainly immature thymocytes
|
|
• in colony assays, bone marrow cells do not respond to IL-7 or to LPS
• in colony assays, the number of myeloid colonies induced by L-cell-conditioned medium containing M-CSF, is reduced 4-10 fold
• decrease in cell numbers and replacement of large areas of hematopoiesis by adipoctyes in the bone marrow
|
|
• number of mature B cells (B220+sIg+) is significantly reduced
|
|
• hypoplasia of the red pulp
|
small spleen
(
J:17712
)
|
• hypoplasia of the white pulp
|
|
• serum IgM levels are reduced to 70% of wild-type levels
|
immune system
|
• proliferative response of adult, but not newborn, splenocytes to LPS or ConA stimulation is reduced 8-10-fold compared to wild-type
|
|
• number of thymocytes is decreased to less than 1% of wild-type
|
|
• involuted thymus
|
|
• absolute number of immature B cells (B220+HSA+ and B220+BPl+) is significantly reduced
|
|
• the immature CD4-CD8- thymocyte population is increased from 4% of total cells in wild-type to 90% of total cells in mutants, although in absolute numbers, this population size is decreased to 12% of wild-type, indicating the presence of mainly immature thymocytes
|
|
• number of mature B cells (B220+sIg+) is significantly reduced
|
|
• hypoplasia of the red pulp
|
small spleen
(
J:17712
)
|
• hypoplasia of the white pulp
|
|
• serum IgM levels are reduced to 70% of wild-type levels
|
|
• moribound mice exhibit pneumonia
|
skeleton
|
• broadening and splitting of the neural arch of the atlas or the axis
|
|
• skeletal transformations affecting vertebrae; incomplete penetrance, although each mutant has at least one transformation and 87% have multiple transformations
|
|
• transformation of T7 to T8, resulting in the presence of six vertebrosternal ribs instead of seven in controls
• transformation of T13 to the first lumbar vertebra (L1), as evidenced by the absence or degeneration of the ribs normally associated with T13
|
|
• transformation of the seventh cervical vertebra (C7) to the first thoracic vertebra (T1), as evidenced by the presence of ribs at C7, which fuse on the ventral side with the ribs associated with T1
|
|
• transformation of L6 to the first sacral vertebra (S1), as evidenced by the association of the ilial bones with L6 instead of S1 as in controls
|
nervous system
|
• major white matter tracts, such as the corpus callosum, show extensive gliosis
|
|
• extensive gliosis
|
|
• dark shrunken and degenerating neurons are seen in some mutants
|
|
• reduced thickness of cerebellar layers, neuron loss
|
|
• shrunken Purkinje cells
|
respiratory system
|
• moribound mice exhibit pneumonia
|
cellular
|
• major white matter tracts, such as the corpus callosum, show extensive gliosis
|
|
• proliferative response of adult, but not newborn, splenocytes to LPS or ConA stimulation is reduced 8-10-fold compared to wild-type
|
endocrine/exocrine glands
|
• number of thymocytes is decreased to less than 1% of wild-type
|
|
• involuted thymus
|
