mortality/aging
• mice are born at the expected Mendelian ratio but die at P1
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craniofacial
• severe hypoplasia and reduced ossification of many neural crest-derived bones at E18.5
• both the shape and size of most neural crest-derived bones including alisphenoid, premaxilla, palatal process of premaxilla, palatal process of maxilla and palatine are affected
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• frontal bones are hypoplastic and widely separated leaving a wide dorsal opening
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• reduced ossification of the nasal bone at E18.5
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• palatal process of palatine bone is missing at E18.5
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• Ki67 immunohistochemistry showed a significant decrease in mesenchymal cell proliferation in middle palatal shelves sections at E15.5
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• palatal shelves are elevated above the tongue to a horizontal position but completely fail to fuse at the midline throughout the anterior-posterior axis
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• all fetuses show a secondary cleft palate defect at E15.5 and E18.5
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skeleton
N |
• no apparent defects in the axial skeleton at E18.5
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• severe hypoplasia and reduced ossification of many neural crest-derived bones at E18.5
• both the shape and size of most neural crest-derived bones including alisphenoid, premaxilla, palatal process of premaxilla, palatal process of maxilla and palatine are affected
|
• frontal bones are hypoplastic and widely separated leaving a wide dorsal opening
|
• reduced ossification of the nasal bone at E18.5
|
• palatal process of palatine bone is missing at E18.5
|
• reduced ossification of the nasal bone and many neural crest-derived bones at E18.5
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growth/size/body
• reduced ossification of the nasal bone at E18.5
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• palatal process of palatine bone is missing at E18.5
|
• Ki67 immunohistochemistry showed a significant decrease in mesenchymal cell proliferation in middle palatal shelves sections at E15.5
|
• palatal shelves are elevated above the tongue to a horizontal position but completely fail to fuse at the midline throughout the anterior-posterior axis
|
• all fetuses show a secondary cleft palate defect at E15.5 and E18.5
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digestive/alimentary system
• palatal process of palatine bone is missing at E18.5
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• Ki67 immunohistochemistry showed a significant decrease in mesenchymal cell proliferation in middle palatal shelves sections at E15.5
|
• palatal shelves are elevated above the tongue to a horizontal position but completely fail to fuse at the midline throughout the anterior-posterior axis
|
• all fetuses show a secondary cleft palate defect at E15.5 and E18.5
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cardiovascular system
N |
• no alterations in smooth muscle actin staining in the aortic arches and normal septation and alignment of the aorta and pulmonary trunk at E17.5, indicating normal cardiac outflow tract development
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endocrine/exocrine glands
N |
• normal thymus and adrenal gland (chromaffin cells) morphology at E17.5
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respiratory system
• reduced ossification of the nasal bone at E18.5
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