behavior/neurological
• mice emit an intermittent, high-pitched chirping sound
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respiratory system
• cartilaginous rings of the trachea are frequently disrupted anteriorly, resembling an arch missing its keystone, thus collapsing the transverse diameter of the tracheal airway
• mean number of disrupted cartilaginous rings is around 9.95
• abnormal tracheal rings are seen at both E14.5 and P1 and almost all the cartilage rings are disrupted at these times, indicating congenital tracheal malformations
• however, cartilage ring pattern in the bronchi, cricoid cartilage and cartilages from the xiphistemum, ear, nose and articular cartilage are normal
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• tracheas are narrow and elliptically shaped, with the long axis oriented in the anterior-posterior direction
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skeleton
• cartilaginous rings of the trachea are frequently disrupted anteriorly, resembling an arch missing its keystone, thus collapsing the transverse diameter of the tracheal airway
• mean number of disrupted cartilaginous rings is around 9.95
• abnormal tracheal rings are seen at both E14.5 and P1 and almost all the cartilage rings are disrupted at these times, indicating congenital tracheal malformations
• however, cartilage ring pattern in the bronchi, cricoid cartilage and cartilages from the xiphistemum, ear, nose and articular cartilage are normal
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• embryonic limb bud mesenchymal cells undergo chondrogenic differentiation in cell culture to a less extent than wild-type mesenchymal cells
• marker analysis indicates that condensation of chondroprogenitor cells in the trachea is disrupted
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