mortality/aging
• die shortly after birth from respiratory failure
|
growth/size/body
• small chest wall
|
• dwarfism with shortened limbs
|
respiratory system
skeleton
• osteoblasts are immature at E18.5, indicating that differentiation is inhibited
• osteoblast development is almost completely blocked in calvarial culture
|
• maturation of chondrocytes into hypertrophic cells is delayed
|
• calcification is poor in endochondral bones, including the chondrocranium, sternum, ribs, vertebrae, and limbs
• endochondral ossification is retarded at E18.5, with more severely retarded ossification in femurs than tibiae
|
• poor intramembranous bone formation
• frontal and parietal bones and maxilla and mandible of the skull are only partially calcified at E18.5
|
hematopoietic system
small thymus
(
J:80668
)
• mutants exhibit a rudimentary thymus composed of epithelial cells
|
• no hematopoiesis in lymphoid lineage
|
• rudimentary thymus does not contain lymphoid cells
• B220+ B lymphocytes are absent in the liver
|
• thymuses virtually lack T cells and their progenitors
|
small spleen
(
J:80668
)
• small spleen composed primarily of epithelial cells and erythroid lineage cells with no B lymphocytes
|
immune system
small thymus
(
J:80668
)
• mutants exhibit a rudimentary thymus composed of epithelial cells
|
• rudimentary thymus does not contain lymphoid cells
• B220+ B lymphocytes are absent in the liver
|
• thymuses virtually lack T cells and their progenitors
|
small spleen
(
J:80668
)
• small spleen composed primarily of epithelial cells and erythroid lineage cells with no B lymphocytes
|
limbs/digits/tail
short limbs
(
J:80668
)
cardiovascular system
N |
• hemorrhaging is not observed as in Cbfb null mice
|
cellular
• osteoblasts are immature at E18.5, indicating that differentiation is inhibited
• osteoblast development is almost completely blocked in calvarial culture
|
endocrine/exocrine glands
small thymus
(
J:80668
)
• mutants exhibit a rudimentary thymus composed of epithelial cells
|