title: Blood group glycosphingolipid expression in kidney of an individual with the rare blood group A1 Le(a-b+) p phenotype: absence of blood group structures based on the globoseries.
abstract: Total neutral glycolipid fractions were isolated from kidney and ureter tissue obtained at autopsy of an individual of the rare blood group A1 Le(a-b+) p. The amount of glycolipids isolated were 3.7 and 2.5 mg g-1 dry tissue weight for the kidney and ureter tissue, which is in the range of reference blood group P kidneys. Part of the kidney glycolipid fraction was subfractionated by HPLC. Glycolipid compounds were structurally characterized by thin-layer chromatography (chemical detection and immunostaining with monoclonal antibodies), proton NMR spectroscopy and mass spectrometry. Globotriaosyl- and globotetraosyl-ceramides, which are the major compounds in kidneys of P individuals, were absent in the p kidney, and a comparatively increased amount of monoglycosyl- and lactosylceramides was found. A shift to longer fatty acyl chains in the ceramide part of lactosylceramides was noted. Elongated globoseries compounds with five to seven sugar residues, including the blood group A type 4 chain structure, were lacking. A slight increase in neolactotetraosyl- and blood group X pentaglycosyl-ceramides was noticed. The study confirms an enzymatic block in the conversion of lactosylceramide to elongated globoseries compounds in the kidney tissue similar to that of erythrocytes of p individuals.
authors: Department of Clinical Chemistry and Transfusion Medicine, University of Göteborg, Sahlgrenska Hospital, Sweden.
authors: K
authors: K
authors: Lindström
authors: P A
authors: PA
authors: Jovall
authors: S
authors: S
authors: Ghardashkani
authors: B E
authors: BE
authors: Samuelsson
authors: M E
authors: ME
authors: Breimer
mesh_terms: D000818:Animals; D000911:Antibodies, Monoclonal; D000918:Antibody Specificity; D001789:Blood Group Antigens / Q000096:biosynthesis* / Q000737:chemistry; D002851:Chromatography, High Pressure Liquid; D002855:Chromatography, Thin Layer; D005260:Female; D005915:Globosides / Q000096:biosynthesis* / Q000737:chemistry; D006017:Glycolipids / Q000096:biosynthesis* / Q000737:chemistry / Q000302:isolation & purification; D006028:Glycosphingolipids / Q000096:biosynthesis* / Q000737:chemistry / Q000302:isolation & purification; D006801:Humans; D007668:Kidney / Q000737:chemistry / Q000378:metabolism*; D009682:Magnetic Resonance Spectroscopy; D013058:Mass Spectrometry; D051379:Mice; D010127:P Blood-Group System / Q000235:genetics*; D010641:Phenotype; D014513:Ureter / Q000737:chemistry / Q000378:metabolism*
chemical_list: D000911:Antibodies, Monoclonal; D001789:Blood Group Antigens; D005915:Globosides; D006017:Glycolipids; D006028:Glycosphingolipids; D010127:P Blood-Group System