mortality/aging
• 32% of mice die by week 2
|
skeleton
• abnormal neural crest derived and thoracic skeleton
|
• 55% of mice have reduced calvarial bones at the midline with increases in the gaps between frontal bones
|
• increases in the gaps between frontal bones
|
• 78% of homozygous mice have smaller presphenoid bone processes that are less extended towards the midline
|
• 78% of homozygous mice have smaller palatine bone processes that are less extended towards the midline
|
• 44% of mice have sternum defects including asymmetric or additional fusion of ribs and gaps in the sternum
|
renal/urinary system
• kidney is swollen in sections and blood filled
|
• glomeruli are often degraded
• excess smooth muscle cells are found in the glomeruli
|
small kidney
(
J:117491
)
pale kidney
(
J:117491
)
hematopoietic system
• by week 6
|
• by week 6
|
• by week 6
|
• an increase in immature red blood cells is observed compared to wild-type mice
|
homeostasis/metabolism
cardiovascular system
hemorrhage
(
J:117491
)
• at E11.5-E18.5
|
muscle
• excess smooth muscle cells are found in the glomeruli
|
immune system
• kidney is swollen in sections and blood filled
|
craniofacial
• 55% of mice have reduced calvarial bones at the midline with increases in the gaps between frontal bones
|
• increases in the gaps between frontal bones
|
• 78% of homozygous mice have smaller presphenoid bone processes that are less extended towards the midline
|
• irregularities in fusion persist into adulthood
|
• 78% of homozygous mice have smaller palatine bone processes that are less extended towards the midline
|
broad snout
(
J:117491
)
• snout is shorter and wider than in wild-type mice
|
short snout
(
J:117491
)
• snout is shorter and wider than in wild-type mice
|
digestive/alimentary system
• irregularities in fusion persist into adulthood
|
• 78% of homozygous mice have smaller palatine bone processes that are less extended towards the midline
|
growth/size/body
• irregularities in fusion persist into adulthood
|
• 78% of homozygous mice have smaller palatine bone processes that are less extended towards the midline
|
broad snout
(
J:117491
)
• snout is shorter and wider than in wild-type mice
|
short snout
(
J:117491
)
• snout is shorter and wider than in wild-type mice
|
respiratory system