title: [Analysis of molecular microheterogeneity of plasma glycoproteins in a patient with sialidosis 1].
abstract: Using specific polyclonal monovalent antibodies, the molecular microheterogeneity of acute phase proteins: orosomucoid, alpha1-antitrypsin and ceruloplasmin, circulating in peripheral blood of a healthy donor and a patient with a hereditary deficiency of lysosomal neuraminidase (syalidosis I or the cherry stone syndrome), was analyzed by use of 2D electrophoresis. The specific distinctions due to a deficiency [caused by a deficiency] of lysosomal neuraminidase were revealed in the population of ceruloplasmin molecules, but not in the molecules of alpha1-antitrypsin and orosomucoid (alpha 1-acid glycoprotein). The molecular genetic bases of molecular microheterogeneity of some plasma glycoproteins and the possible use of natural models in studies of GP's functional role and the pathways of its transfer after internalization into non-hepatocytic (?) cells are discussed.
authors.affiliation: Institute of Experimental Medicine RAMS, St. Petersburg, Russia.
authors.forename: E T
authors.initials: ET
authors.lastname: Zakharova
authors.forename: L V
authors.initials: LV
authors.lastname: Puchkova
authors.forename: M M
authors.initials: MM
authors.lastname: Shavlovskiĭ
authors.forename: M V
authors.initials: MV
authors.lastname: Neuĭmina
authors.forename: E P
authors.initials: EP
authors.lastname: Ruzhdiĭ
authors.forename: V S
authors.initials: VS
authors.lastname: Gaĭtskhoki
mesh_terms: D015180:Electrophoresis, Gel, Two-Dimensional; D006023:Glycoproteins / Q000097:blood*; D006801:Humans; D016464:Lysosomal Storage Diseases / Q000097:blood*; D008297:Male; D009439:Neuraminidase / Q000172:deficiency*
chemical_list: D006023:Glycoproteins; D009439:Neuraminidase
vernacular_title: Analiz molekuliarnoĭ mikrogeterogennosti nekotorykh glikoproteinov plazmy krovi bol'nogo sialidozom 1.