title: [Elevated galactose transport into cells as the cause of development of hereditary galactosemia in rats].
abstract: A strain of rats with symptoms of inherited galactosemia (cataracts, hepatosplenomegaly, aminoaciduria etc) was produced by selection and inbreeding of Wistar rats highly susceptible to the galactosemic effect of galactose. The salient biochemical feature of these rats, like human galactosemics, is manifested as a decrease in the activity of galactose-I-phosphate uridyltransferase (Gal-I-PUT) in liver tissue and erythrocytes. However, the cross experiments have shown that the decrease in Gal-I-PUT activity was not required for expression of main galactosemia symptoms. Genetic analysis of cataract formation demonstrated that this trait was controlled by a single dominant gene. High transport rate of 14C-galactose into erythrocytes was a characteristic of galactosemic rats. Genetic analysis demonstrated that this trait was under the control of a single dominant gene, similar to the cataract formation. The intracellular accumulation of galactose ensured by its high transport, simultaneously with a decrease in Gal-I-PUT activity, were assumed to be the main reasons of galactosemic symptoms. The glucose transporter isolated from erythrocytes of the galactosemic rats, when integrated into the liposome membrane transferred more actively galactose into the liposomes than that of the control galactose resistant rats.
authors.forename: N A
authors.initials: NA
authors.lastname: Solov'eva
authors.forename: E Kh
authors.initials: EKh
authors.lastname: Ginzburg
authors.forename: F S
authors.initials: FS
authors.lastname: Kazarinova
authors.forename: V V
authors.initials: VV
authors.lastname: Kandaurov
authors.forename: R I
authors.initials: RI
authors.lastname: Salganik
mesh_terms: D000818:Animals; D001693:Biological Transport, Active; D004910:Erythrocyte Membrane / Q000378:metabolism; D004912:Erythrocytes / Q000378:metabolism*; D005690:Galactose / Q000378:metabolism*; D005693:Galactosemias / Q000209:etiology* / Q000235:genetics / Q000378:metabolism; D006601:Hexoses / Q000378:metabolism; D051381:Rats; D011922:Rats, Mutant Strains; D013045:Species Specificity
chemical_list: D006601:Hexoses; D005690:Galactose
vernacular_title: Povyshennyĭ transport galaktozy v kletki kak prichina razvitiia nasledstvennoĭ galaktozemii u krys.