[Hereditary deficiency of alpha 1- antitrypsin in rats due to evolving chronic lung pathology].
W/SSM rats which are characterized by hereditary abnormal changes in the lungs, hepato- and splenomegalia and some other disturbances have also alpha 1-antitrypsin (AAT) deficiency. A study of AAT in these rats by means of isoelectrofocusing and immunoblotting with anti-AAT antibodies labelled with peroxidase has demonstrated that deficiency of the protease inhibitor is not associated with any disturbances of its synthesis or any changes of its electrophoretic properties. A higher activity of lysosomal glycosidases and proteinases was found in the liver and leukocytes of W/SSM rats. It is suggested that AAT deficiency is due to its modification under the influence of lysosomal enzymes. The described biochemical distances seem to be associated with an increased hexose transport into the cells, which is controlled by a mutant gene.
Soloveva
N A
NA
Grishaeva
O N
ON
Parik
Iu Ia
IuIa
Kosova
E Iu
EIu
Korolenko
T A
TA
D000818:Animals; D000906:Antibodies / Q000097:blood; D002908:Chronic Disease; D006026:Glycoside Hydrolases / Q000032:analysis; D008099:Liver / Q000378:metabolism* / Q000473:pathology; D008168:Lung / Q000378:metabolism* / Q000473:pathology; D008171:Lung Diseases / Q000235:genetics* / Q000378:metabolism* / Q000473:pathology; D008247:Lysosomes / Q000201:enzymology / Q000473:pathology; D008297:Male; D051381:Rats; D019896:alpha 1-Antitrypsin Deficiency
D000906:Antibodies; D006026:Glycoside Hydrolases