HEREDITARY ALPHA(1)-ANTITRYPSIN DEFICIENC Y IN RATS DUE TO DEVELOPMENT OF CHRONIC LUNG-DISEASE

W/SSM rats which are characterized by hereditary abnormal changes in t he lungs, hepato- and splenomegalia and some other disturbances have a lso alpha1-antitrypsin (AAT) deficiency. A study of AAT in these rats by means of isoelectrofocusing and immunoblotting with anti-AAT antibo dies labelled with peroxidase has demonstrated that deficiency of the protease inhibitor is not associated with any disturbances of its synt hesis or any changes of its electrophoretic properties. A higher activ ity of lysosomal glycosidases and proteinases was found in the liver a nd leukocytes of W/SSM rats. It is suggested that AAT deficiency is du e to its modification under the influence of lysosomal enzymes. The de scribed biochemical distances seem to be associated with an increased hexose transport into the cells, which is controlled by a mutant gene.