mortality/aging
• at 1 week of age no pups are found
|
• only 7% of pups at P1 are homozygous rather than the expected 25%
|
renal/urinary system
kidney cyst
(
J:108109
)
• sporadic cysts at E17.5 sometimes occurring in Bowman's space
|
• the number of collecting ducts is reduced to 8 +/- 1 and 9 +/- 2 compared to 20 +/- 2 and 28 +/- 2 in wild-type mice at E14.5 and E16.5, respectively
|
• abnormal clustering of the glomeruli
|
• at E12.0, E14.5, and E16.5 increased cell death is seen in thickened areas of the mesenchyme at the periphery of the kidney; however around the collecting duct tips cell death is similar to wild-type
• at E12.5 expression patterns indicate that few foci of nephrogenic mesenchyme condensations are formed, the stromal cell population is increased, the number of epithelial structures is reduced, and the normal segregation of stoma and mesenchyme is incomplete
• at E12.5 the cortical cell layer is thicker than in wild-type and increased cell death is seen in the cortical regions
• at E14.5 the cell layer between the nephrogenic mesenchyme and the kidney capsule is thicker than in wild-type
• at E14.5 and E16.5 the tips of the collecting ducts are surrounded by variably reduced amounts of nephrogenic mesenchyme with about 50% lacking any surrounding nephrogenic mesenchyme
|
small kidney
(
J:108109
)
• about half the volume of wild-type kidneys at E17.5
|
hydroureter
(
J:108109
)
• at E17.5
|
growth/size/body
kidney cyst
(
J:108109
)
• sporadic cysts at E17.5 sometimes occurring in Bowman's space
|