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Related Experiment Videos

Hereditary emphysema in the tight-skin (Tsk/+) mouse

S V Szapiel, J D Fulmer, G W Hunninghake

    The American Review of Respiratory Disease
    |June 1, 1981
    PubMed
    Summary

    The tight-skin (Tsk/+) mouse model displays lung and thoracic abnormalities consistent with emphysema. This genetic model exhibits enlarged air spaces and altered lung mechanics, offering insights into destructive lung disease.

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    Area of Science:

    • Pulmonary Medicine
    • Genetics
    • Pathology

    Background:

    • The tight-skin (Tsk/+) mouse exhibits an autosomal dominant mutation causing increased thoracic size and lung volume.
    • Abnormalities in connective tissue, cartilage, tendon, and bone are also observed in Tsk/+ mice.

    Purpose of the Study:

    • To investigate if the Tsk/+ mouse model displays morphologic and physiologic characteristics of emphysema.
    • To determine the utility of the Tsk/+ mouse as a model for studying the pathogenesis of destructive lung disease.

    Main Methods:

    • Light and scanning electron microscopy were used to examine lung morphology in Tsk/+ and normal mice.
    • Pulmonary function tests were conducted to assess lung mechanics, including total lung capacity, compliance, and specific compliance.

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    Main Results:

    • Tsk/+ mouse lungs showed generalized enlargement of air spaces, subpleural cysts, and bullae.
    • Alveolar walls were thinned or broken, with increased pores of Kohn.
    • Tsk/+ mice exhibited significantly increased total lung capacity, compliance, and specific compliance compared to normal mice.

    Conclusions:

    • The Tsk/+ mouse is a genetically determined model of emphysema.
    • This model may be valuable for investigating the pathogenesis of destructive lung diseases like emphysema.