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• W1516307532 abstract "The tight-skin mouse is a newly described mutant characterized in heterozygotes (Tsk/ +) by generalized connective tissue defects. Since the Tsk/ + mouse also has an enlarged thorax with enlarged lungs, features often found in people with destructive lung diseases, this animal was evaluated as a possible model of pulmonary emphysema. Morphologically, Tsk/ + mouse lungs are characterized by enlarged airspaces, distention of alveoli, and destruction of alveolar septa. In addition, there are increased numbers of pores of Kohn which appear irregular in size. Physiologically, Tsk/ + lungs have an increased total lung capacity and an increased lung compliance compared to normal mice. The destructive lung disease in the Tsk/ + mice is associated with increased numbers of alveolar macrophages and neutrophils at sites of disease activity. The presence of neutrophils in the lungs of Tsk/ + mice was confirmed by bronchoalveolar lavage. The proportion of lung T- and B-lymphocyte sub-populations in Tsk/ + mice was similar to that present in normal animals. The antiprotease screen of the Tsk/ + mice seems to be normal since the ability of their serum to inhibit neutrophil elastase is similar to that of control mice. Thus, the Tsk/ + mouse is a genetically determined model of pulmonary emphysema associated with increased numbers of protease secreting cells in the alveolar structures and a generalized connective tissue abnormality. This model may be useful to understand the interaction between connective tissue defects, increased protease activity in the lung, and destruction of alveolar capillary units." @default.
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• W1516307532 date "1981-01-01" @default.
• W1516307532 modified "2023-09-23" @default.
• W1516307532 title "THE TIGHT-SKIN MOUSE : AN ANIMAL MODEL OF INHERITED EMPHYSEMA" @default.
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• W1516307532 doi "https://doi.org/10.1016/b978-0-08-027379-2.50016-8" @default.
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