Na. Solovyeva et al., HEREDITARY ALPHA(1)-ANTITRYPSIN DEFICIENC Y IN RATS DUE TO DEVELOPMENT OF CHRONIC LUNG-DISEASE, VESTNIK ROSSIISKOI AKADEMII MEDITSINSKIKH NAUK, (2), 1994, pp. 31-34
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.