Analysis Tools
mortality/aging
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• some die spontaneously during the early postnatal period
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muscle
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• hypertrophy is most likely a consequence of severe lung alterations, eventually leading to a cor pulmonale
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• hypertrophy is most likely a consequence of severe lung alterations, eventually leading to a cor pulmonale
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growth/size/body
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• relative weight of the heart is increased after 3 weeks of age, with a 29% higher relative heart weight at 9 weeks of age
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• seen only in older mutants with severe pulmonary remodeling
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• hypertrophy is most likely a consequence of severe lung alterations, eventually leading to a cor pulmonale
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• hypertrophy is most likely a consequence of severe lung alterations, eventually leading to a cor pulmonale
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• relative weight of ovaries is increased
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• absolute and relative carcass weight is reduced
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• exhibit reduced body weight gain from early postnatal life
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• reduction in nose-rump length
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• absolute and relative weight of lung is increased
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• relative weight of the liver is increased
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• absolute and relative weight of spleen is increased
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cardiovascular system
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• thickening of the atrioventricular valves, particularly the mitral valve
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• thickening of the mitral valve
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• hearts are round shaped
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• relative weight of the heart is increased after 3 weeks of age, with a 29% higher relative heart weight at 9 weeks of age
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• seen only in older mutants with severe pulmonary remodeling
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• hypertrophy is most likely a consequence of severe lung alterations, eventually leading to a cor pulmonale
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• hypertrophy is most likely a consequence of severe lung alterations, eventually leading to a cor pulmonale
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• progressive development of alveolar hemorrhage, with small hemorrhagic foci first seen at 2-3 days of age
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skeleton
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• skull is reduced in length at 9 weeks of age
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• width of skull is increased, especially in the region formed by parietal and interparietal bones
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• exhibit overall disproportionate skeletal growth with absolute widths of bones such as the humerus, femur, and skull significantly different
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vision/eye
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• absolute and relative weight of eyes is increased
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• the lateral and anterior part of the lens is disrupted by strong intercellular clefts and vacuoles
• the lens bow structure that is formed by lens epithelial nuclei curving into the cortex is absent
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• remaining lens fiber cells in the nonvacuolated center and posterior region are severely swollen and irregularly arranged
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• remaining retinal layers have an abnormal shape
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• ganglion cell layer does not show a consistent line of cells as in controls
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• the inner nuclear layer shows a disrupted lamination with a wave-like structure, varies from 1-7 cell layers, and shows thinning
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• retina is about half the normal thickness
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respiratory system
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• progressive development of alveolar hemorrhage, with small hemorrhagic foci first seen at 2-3 days of age
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• exhibit nodular pulmonary remodeling, consisting of multiple foci composed of fibroblasts, collagen fibers, large macrophages, and some proteinaceous deposits
• starting at 2-3 days of age, exhibit progressive development of pulmonary lesions, showing macrophages undergoing erythrophagocytosis and granulocytic inflammation
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• absolute and relative weight of lung is increased
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• distal airspaces are almost completely filled with macrophages and show accumulations of eosinophilic and neutrophilic granulocytes
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• distal air spaces are severely enlarged with little septation
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• commonly observe respiratory distress in adults, characterized by rapid and short breathing movements
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endocrine/exocrine glands
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• pancreas weight is disproportionately decreased to body weight
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• relative weight of ovaries is increased
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hematopoietic system
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• absolute and relative weight of spleen is increased
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homeostasis/metabolism
immune system
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• absolute and relative weight of spleen is increased
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liver/biliary system
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• relative weight of the liver is increased
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reproductive system
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• relative weight of ovaries is increased
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craniofacial
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• skull is reduced in length at 9 weeks of age
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• width of skull is increased, especially in the region formed by parietal and interparietal bones
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