mortality/aging
• 8% of mice die by week 1
|
skeleton
• abnormal neural crest derived and thoracic skeleton
|
• 73% of homozygous mice have smaller presphenoid bone processes that are less extended towards the midline
|
• 73% of homozygous mice have smaller palatine bone processes that are less extended towards the midline
|
• 27% of mice have sternum defects including asymmetric or additional fusion of ribs and gaps in the sternum
|
• 47% of mice have a 14th pair of ribs
|
• 33% of mice have widened vertebral arches and forked vertebrae
|
• 33% of mice have widened vertebral arches and forked vertebrae
|
renal/urinary system
• glomeruli are often degraded
• smooth muscle cell number is reduced in the glomeruli
• migration of smooth muscle cells in glomeruli is impaired
|
small kidney
(
J:117491
)
pale kidney
(
J:117491
)
• decrease in kidney function
|
• kidney is swollen in sections and blood filled
|
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
muscle
• smooth muscle cell number is reduced in the glomeruli
|
cardiovascular system
hemorrhage
(
J:117491
)
• at E11.5-E18.5
|
immune system
• kidney is swollen in sections and blood filled
|
cellular
• migration of smooth muscle cells in glomeruli is impaired
|
craniofacial
• 73% of homozygous mice have smaller presphenoid bone processes that are less extended towards the midline
|
• irregularities in fusion persist into adulthood
|
• 73% of homozygous mice have smaller palatine bone processes that are less extended towards the midline
|
digestive/alimentary system
• irregularities in fusion persist into adulthood
|
• 73% of homozygous mice have smaller palatine bone processes that are less extended towards the midline
|
growth/size/body
• irregularities in fusion persist into adulthood
|
• 73% of homozygous mice have smaller palatine bone processes that are less extended towards the midline
|