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OBJECTIVE To study the development of emphysema in transgenic mice. METHODS From the constructed transgenic mice, the reverse phase high performance liquid chromatography (HPLC) analysis was carried out; the P37/P32 ratios were compared; hematoxylin and eosin and Verhoeff Van Giesen histopathologic staining was carried out on paraffin embedded tissue sections. RESULTS Under the transcriptional control of 3 2 kb of the promoter of the rat tropoelastin gene, expression of a minigene which lacks the conserved hydrophobic domain encoded by exon 33 resulted in the progressive development of a lethal pulmonary disorderresembling an emphysematous phenotype in multiple founder lines of transgenic mice. These mice often did prematurely and histological analysis of the lung revealed marked enlargement of alveoli, sepal damage, and fragment sepal elastic fibers. CONCLUSION The development of emphysema in the transgenic animals is the first example of an elastic tissue disorder due directly to aberrant expression of the tropoelastin gene. Our results suggest that additional disease phenotypes may manifest as a result of alteration in the expression of tropoelastin and tropoelastin gene mutations, therefore, will be result in heritable disorders of elastic tissue.
OBJECTIVE To study the development of emphysema in transgenic mice. The reverse phase high performance liquid chromatography (HPLC) analysis was carried out; the P37 / P32 ratios were compared; hematoxylin and eosin and Verhoeff Van Giesen histopathologic staining RESULTS Under the transcriptional control of 32 kb of the promoter of the rat tropoelastin gene, expression of a minigene which lacks the conserved hydrophobic domain encoded by exon 33 resulted in the progressive development of a lethal pulmonary disorderresembling an emphysematous phenotype in multiple founder lines of transgenic mice. These mice often did prematurely and histological analysis of the lung revealed marked enlargement of alveoli, sepal damage, and fragment sepal elastic fibers. CONCLUSION The development of emphysema in the transgenic animals is the first example of an elastic tissue disorder due directly to ab errant expression of the tropoelastin gene. Our results suggest that additional disease phenotypes may manifest as a result of alteration in the expression of tropoelastin and tropoelastin gene mutations, therefore, will be result in heritable disorders of elastic tissue.