Analysis Tools
mortality/aging
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• 13% of mice die between 21 and 25 days of birth
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• 75% of mice die within 24 hours of birth of respiratory distress
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skeleton
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• dead or dying newborns have sternum defects
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• one mouse had sternebrae that were not properly aligned along the ventral midline
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• 38% of mice have severely split sternum, 34% of mice have two sternal bands that are close but have not fused, and 3 of 29 mice exhibited partial fusion of the sternal bands
(J:37682)
• at birth, some mice exhibit varying degrees of split sternum
(J:71389)
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• in 76% of mice the anterior arch is slanted rostrally
• 11 of 22 mice with the rostrally slanted arch the anterior arch is almost parallel with the axial skeleton, a phenotype not observed in wild-type mice, whereas more severe phenotypes the tilt is only a 45 degree with respect to the vertebral column
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• in 55% of mice a C2 to C1 transformation occurs in which the neural arch of the axis of C2 is broadened and in some cases an ectopic anterior arch on the ventral side is present
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• the neural arch of the axis is broadened in the C2 to C1 transformation
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nervous system
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• facial nuclei are undetected in 6 of 6 mice
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• the VII cranial nerve is reduced in 5 of 6 mice
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• in 1 of 6 mice
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behavior/neurological
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• at 3 weeks, surviving mice exhibit severe paralysis of the facial muscles such that mice cannot move their whiskers and nose, did not close their eyes in response to touch and failed to move their ears in response to touch or noise
• facial paralysis is more severe than in Hoxd1 mice
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• females that survive and reproduce have difficulties raising their pups
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craniofacial
narrow face
(
J:37682
)
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• 10 days, mice have narrow faces that become progressively more severe with age
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• at 3 weeks of age, the levator nasolabialis and levator abii maxillaries is absent
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• at 3 weeks of age, the levator nasolabialis and levator labii maxillaris are absent and remaining facial muscles are reduced in mass 2-fold
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• at 3 weeks, surviving mice have receded lower lips
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vision/eye
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• mice cannot close eyelids
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respiratory system
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• 75% of mice die within 24 hours of birth of respiratory distress
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muscle
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• at 3 weeks of age, the levator nasolabialis and levator abii maxillaries is absent
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• at 3 weeks of age, the levator nasolabialis and levator labii maxillaris are absent and remaining facial muscles are reduced in mass 2-fold
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growth/size/body
narrow face
(
J:37682
)
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• 10 days, mice have narrow faces that become progressively more severe with age
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• at 3 weeks of age, the levator nasolabialis and levator abii maxillaries is absent
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• at 3 weeks of age, the levator nasolabialis and levator labii maxillaris are absent and remaining facial muscles are reduced in mass 2-fold
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• at 3 weeks, surviving mice have receded lower lips
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• at E13.5, some mice exhibit a thin body wall compared to wild-type mice and the lateral extent of the thinning region is variable
• thinning of the body wall is similar to that seen in Hoxb4tm1Bay mice but is less extensive
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• at 3 weeks, surviving mice are runted
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