mortality/aging
• homozygous pups die within a few minutes after birth from respiratory failure
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cellular
• multiple large blebs are seen in 38-39% of homozygous null primary MEF nuclei
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• homozygous null primary MEFs fail to differentiate under culture conditions in which wild-type and heterozygous primary MEFs differentiate
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• homozygous null primary MEFs display reduced proliferative potential in culture, reaching proliferative crisis sooner compared to wild-type and heterozygous primary MEFs
• more than 80% of homozygous null primary MEFs were polyploid after 4 passag
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craniofacial
• the skull appears flatter than normal
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• the coronal suture is narrowed and the sagital suture obscured by bone overlap
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• the sagital suture obscured by bone overlap
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embryo
• embryos are smaller than normal
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growth/size/body
• embryos are smaller than normal
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respiratory system
• the lungs are abnormal in 9 out of 11 embryos at E18.5
• the mesenchymal tissue is thicker compared to wild-type mice
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• in 2 of the embryos (out of 11) no alveoli are seen
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• homozygous pups die within a few minutes after birth from respiratory failure
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skeleton
• the skull appears flatter than normal
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• the coronal suture is narrowed and the sagital suture obscured by bone overlap
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• the sagital suture obscured by bone overlap
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• distortion of the thoracic vertebral column is seen
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• embryos display an abnormal curvature of the spine
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• at E18.5 reduced Alizarin red staining of many bones suggests delayed or impaired ossification
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