mortality/aging
• though present in Mendelian ratios at E18.5, mice no viable mice are born
• mice delivered by Caesarian die within an hour of birth
|
renal/urinary system
N |
• despite defects in the ureters, mice exhibit a normal bladder
|
• explants of E15.5 ureters completely fail to exhibit peristaltic contraction unlike wild-type explants
|
• mice exhibit dilation of the renal pelvis
|
• from E16.5, mice exhibit bilateral hydronephrosis
• however, no physical obstructions are detected
|
• the structural organization of muscular layers is lost leading to thin layers of mesenchymal cells unlike in wild-type mice
• however, the distal ureters are normal and mesenchymal progenitors condense normally until E15.5
• at E16, peri-urothelial cells of ureters are less organized than in wild-type mice
|
• development of proximal ureteric smooth muscle cells is impaired as determined by expression of smooth muscle markers
• however, proliferation and apoptosis of periureteric cells, differentiation of uroepithelium, and development of stromal cells are normal
|
• development of proximal ureteric smooth muscles is impaired as determined by expression of smooth muscle markers
• however, smooth muscle development in the bladder and proliferation and apoptosis of ureteric smooth muscle precursors are normal
• the structural organization of muscular layers is lost leading to thin layers of mesenchymal cells unlike in wild-type mice
|
• mice exhibit dilated proximal ureters with thin walls
|
hydroureter
(
J:138769
)
• from E16.5, mice exhibit prominent bilateral proximal hydroureter
• however, no physical obstructions are detected
|
respiratory system
atelectasis
(
J:138769
)
• lungs fail to inflate as indicated by their sinking instead of floating on water as do wild-type lungs
|
• at birth, mice do not breath
|
behavior/neurological
• at birth, mice only respond to strong stimuli
|
• at birth
|
muscle
• explants of E15.5 ureters completely fail to exhibit peristaltic contraction unlike wild-type explants
|