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.

• 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.

Ämnesord

MEDICIN OCH HÄLSOVETENSKAP  -- Klinisk medicin -- Kirurgi (hsv//swe)

MEDICAL AND HEALTH SCIENCES  -- Clinical Medicine -- Surgery (hsv//eng)

Nyckelord

Animals

Antibodies

Monoclonal

Antibody Specificity

Blood Group Antigens

biosynthesis

chemistry

Chromatography

High Pressure Liquid

Chromatography

Thin Layer

Female

Globosides

biosynthesis

chemistry

Glycolipids

biosynthesis

chemistry

isolation & purification

Glycosphingolipids

biosynthesis

chemistry

isolation & purification

Humans

Kidney

chemistry

metabolism

Magnetic Resonance Spectroscopy

Mass Spectrometry

Mice

P Blood-Group System

genetics

Phenotype

Ureter

chemistry

metabolism

Publikations- och innehållstyp

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