Analysis Tools
mortality/aging
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• ~80% of mice die between birth and weaning, with less than 20% homozygotes (versus expected 25%) obtained by postnatal week 1 (PW1), only ~17% by PW2, and ~6% by PW3
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growth/size/body
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• at birth, mice weigh 18.4% less than controls
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• at PW6, mice are obviously smaller than controls
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• mice exhibit progressive growth retardation from birth to PW6
• mice weigh 22% less than controls at PW2, 36% less at PW3, and 48% less at PW4
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limbs/digits/tail
polydactyly
(
J:222441
)
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• all mice exhibit duplication of digit 1 in all four limbs
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cellular
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• mice exhibit widespread ciliation defects
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• many of the motile cilia formed in the ventral node at E8.0 are shorter than wild-type cilia
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• fewer motile cilia are present in the ventral node at E8.0 relative to wild-type controls
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• primary cilia present in the central canal of the spinal neural tube at E10.5 are shorter than wild-type cilia
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• at PW6, mice show a 40% reduction in the number of ciliated cells in the kidneys relative to wild-type controls
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skeleton
| N |
• at P0, mice show normal planar cell polarity in proximal tibia and metatarsal growth plates, with proliferating chondrocytes exhibiting a similar flattened morphology and columnar arrangement as wild-type controls
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• at E14.5, long bones (e.g. ulna) display drastically narrower hypertrophic zones than wild-type bones
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• at E17.5 and E18.5, mice exhibit severe rib misalignment
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• 3 of 8 mice show an unequal number of ribs on each side of the sternum (8 attached ribs on the left and 7 attached ribs on the right)
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• ossification of the sternum is delayed
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• at P0, cilia are significantly reduced in number in growth plate chondrocytes in both the proximal tibia and metatarsals
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• ossification within the phalanges is delayed by ~1 day relative to wild-type controls at E16.5, E17.5, and E18.5
• ossification of thoracic vertebrae is reduced and does not extend as caudally as in wild-type mice at E16.5, but this difference is resolved by E17.5 and E18.5
• ossification of the sternum and long bones of the forelimbs is also delayed
• at E16.5, the ossified fraction of long bones is significantly reduced relative to wild-type controls (32% less in humerus, 11% less in radius, 8.3% less in ulna)
• at E17.5, a significant difference is noted in humerus (11% less), but not in radius or ulna
• at E18.5, significantly less ossification is found in radius (9.9% less), but not in humerus or ulna
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nervous system
| N |
• at E10.5, embryos show normal dorsal/ventral patterning, proliferation, and differentiation in the neural tube relative to wild-type controls
• at E18.5, embryos show normal polarity of hair cells in the organ of Corti
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• primary cilia present in the central canal of the spinal neural tube at E10.5 are shorter than wild-type cilia
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exencephaly
(
J:222441
)
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• very rare; only 2 of 202 mice display exencephaly
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embryo
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• many of the motile cilia formed in the ventral node at E8.0 are shorter than wild-type cilia
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• fewer motile cilia are present in the ventral node at E8.0 relative to wild-type controls
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• primary cilia present in the central canal of the spinal neural tube at E10.5 are shorter than wild-type cilia
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renal/urinary system
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• at PW6, mice show a 40% reduction in the number of ciliated cells in the kidneys relative to wild-type controls
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