mortality/aging
• 11% of mice die between weaning and 6 months of age
|
skeleton
• at P21, mice exhibit a smaller than normal skeleton
|
• 5 of 22 mice exhibit incomplete closure of the basisphenoid bone
|
• some mice develop misaligned and over-grown incisors unlike in heterozygous mice
|
• some mice develop misaligned and over-grown incisors unlike in heterozygous mice
|
• 2 of 29 mice exhibit fusion of the anterior tips of the left and right mandible
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short radius
(
J:130163
)
• at E16.5, radius length is 89% of double heterozygotes
• at E18.5, the length of the radius is decreased to 93% of double heterozygotes
• at P21, the length of the radius is reduced to 91% of wild-type
|
• at E16.5, mice exhibit a decrease in the length of the proliferating zone to 90% of double heterozygotes
• however, the ossified and hypertrophic zones were normal in length and there is no difference in the rate of proliferation within the zone
|
• during development mice exhibit a reduction in the number of undifferentiated chondrocytes between sternebra 3 and 4 compared to in double heterozygotes indicating an acceleration of chondrocyte differentiation
|
• at E18.5, 85% of mice exhibit small bony bridges to broad connections between 2 or 3 sternebra
• at P0/P1, 100% of mice exhibit different degrees of sternebrae fusion that never involves more than 3 sternebrae
• 94% of mice exhibit fusion of sternebra 3 and 4 (65% complete) and 18% of mice exhibit a fusion of sternebra 2 and 3
|
• at E18.5, sternum length is decreased 94% of double heterozygotes
• at P21, sternum length is decreased 87% of wild-type
|
wide sternum
(
J:130163
)
• at P21, mice exhibit a broadened sternum compared to in wild-type mice
|
• at P21, mice exhibit a small ribcage
|
• after P21, 94% of mice exhibit calcified fusion of up to 7 distal tail vertebrae
|
• 9 of 29 mice exhibit dorsally malformed second cervical vertebra
|
• 3 mice exhibit transformation of C2 into C1
|
• at P0/P1, mice exhibit fusion of ossification centers in the lumbar vertebrae
|
• at E18.5, 92% of mice exhibit connections in 1 to 5 vertebrae anterior to S2
• at P0/P1, all mice exhibit fusion of ossification centers in the sacral vertebrae
|
• at P21, 88% of mice exhibit split dorsal vertebrae compared to 22% of wild-type mice with 47% of mice exhibiting involvement of 2 to 6 vertebrae
|
craniofacial
• 5 of 22 mice exhibit incomplete closure of the basisphenoid bone
|
• some mice develop misaligned and over-grown incisors unlike in heterozygous mice
|
• some mice develop misaligned and over-grown incisors unlike in heterozygous mice
|
• 2 of 29 mice exhibit fusion of the anterior tips of the left and right mandible
|
growth/size/body
• some mice develop misaligned and over-grown incisors unlike in heterozygous mice
|
• some mice develop misaligned and over-grown incisors unlike in heterozygous mice
|
• at P21, occasionally strongly dwarfed mice are observed
|
• at P0, body weight is reduced to 91% of double heterozygote weights
• at P21, mice weight 70% of double heterozygotes
• however, surviving mice recover to normal weight during postnatal development
|
limbs/digits/tail
short radius
(
J:130163
)
• at E16.5, radius length is 89% of double heterozygotes
• at E18.5, the length of the radius is decreased to 93% of double heterozygotes
• at P21, the length of the radius is reduced to 91% of wild-type
|
• after P21, 94% of mice exhibit calcified fusion of up to 7 distal tail vertebrae
|
kinked tail
(
J:130163
)