mortality/aging
• 43 of 53 mice die at birth
|
• most of the 10 survivors die by P14
|
respiratory system
• 43 of 53 die from respiratory failure after cesarean section
|
growth/size/body
• severe dwarfism at P7 and P14
|
• similar body weight at birth but decreased by 50% at P7 and P14
|
skeleton
• in tibial growth plates at E18.5 and P14
|
• in tibial growth plates at E18.5 and P14
|
• at E18.5
|
short tibia
(
J:256718
)
• at E18.5
|
• smaller
|
• Safranin-O staining indicates drastically decreased glycosaminoglycan content
• severely reduced chondroitin sulfate content
|
• drastically disrupted at P14
|
• drastically disrupted at P14
|
• at P14 proliferating chondrocytes in long bones are round and lack the longitudinal organization seen in controls
• some chondrocytes with a flat shape are seen outside the proliferative zone
|
• delayed formation of secondary ossification centers
|
cellular
• in tibial growth plates at E18.5 and P14
|
• in tibial growth plates at E18.5 and P14
|
limbs/digits/tail
• at E18.5
|
short tibia
(
J:256718
)
• at E18.5
|