The pallid mouse. A model of genetic alpha 1-antitrypsin deficiency.
Laboratory investigation; a journal of technical methods and pathology, 1993•europepmc.org
Background The current hypothesis of pulmonary emphysema is based on an alteration of
the protease-antiprotease balance within the lower respiratory tract. This hypothesis derives
largely from studies in emphysema patients with genetic deficiency in serum alpha 1-
antitrypsin. In animals, naturally occurring deficiency in serum elastase inhibitory capacity
associated with early development of emphysema has been reported in the tight-skin
mouse. We describe here a mouse model of genetic deficiency of alpha 1-antitrypsin in …
the protease-antiprotease balance within the lower respiratory tract. This hypothesis derives
largely from studies in emphysema patients with genetic deficiency in serum alpha 1-
antitrypsin. In animals, naturally occurring deficiency in serum elastase inhibitory capacity
associated with early development of emphysema has been reported in the tight-skin
mouse. We describe here a mouse model of genetic deficiency of alpha 1-antitrypsin in …
Background
The current hypothesis of pulmonary emphysema is based on an alteration of the protease-antiprotease balance within the lower respiratory tract. This hypothesis derives largely from studies in emphysema patients with genetic deficiency in serum alpha 1-antitrypsin. In animals, naturally occurring deficiency in serum elastase inhibitory capacity associated with early development of emphysema has been reported in the tight-skin mouse. We describe here a mouse model of genetic deficiency of alpha 1-antitrypsin in which emphysema occurs late in life.
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