mortality/aging
• although present in Mendelian ratios at E18.5, all mice die prior to weaning
|
muscle
• at E14.5, muscle mass and myoblast fusion are decreased compared to in wild-type mice
• in culture, myoblasts are unable to form long nucleated fibers after 4 days unlike wild-type cells
• however, myoblast differentiation and migration of muscle progenitors are normal
|
• at E12.5, E13.5 and E18.5, the diameter of muscle fibers is decreased compared to in wild-type mice
|
• at E14.5 and E16.5, the attachment of the diaphragm to the intercostal muscles is severely impaired
|
• at E14.5, and E16.5
|
• intercostal muscles are reduced in size leading to ribs stacked closer together than in wild-type mice
|
• at E16.5 and E18.5, the size of deep back muscles, tongue and limb muscles is reduced compared to in wild-type mice
|
• at E13.5, apoptosis of myofibers is increased compared to in wild-type mice
|
respiratory system
atelectasis
(
J:143342
)
• lungs do not inflate
|
growth/size/body
• at birth, mice are smaller than normal and fail to straighten
|
homeostasis/metabolism