Characterization of SSEA-1 glycolipids from the brain of a patient with fucosidosis.
Neutral glycolipids from the brain of a patient with Fucosidosis were analyzed and two complex glycolipids containing five and eight sugars were isolated from the cortical grey matter. These two glycolipids reacted with antibodies recognizing the SSEA-1 [Le(x)(X)] carbohydrate determinant. SSEA-1 glycolipids are normally expressed in human embryonic brain but are found in only small amounts in postnatal human brain. The accumulation of the two SSEA-1 glycolipids in Fucosidosis brain thus represents a defect which affects the normal developmentally regulated decrease in postnatal expression of these glycolipids, and may be a contributing factor in the abnormal brain development associated with the disease. Chemical characterization of the two isolated glycolipids by gas chromatographic and mass spectrometric analyses has identified the two glycolipids as lacto-N-fucopentaosylceramide (III) and difucosyl-neolactonorhexaosylceramide.
Schwarting
G A
GA
Department of Biochemistry, E. K. Shriver Center, Waltham, MA 02254.
Williams
M A
MA
Evans
J E
JE
McCluer
R H
RH
D001923:Brain Chemistry; D002236:Carbohydrate Conformation; D002240:Carbohydrate Sequence; D002241:Carbohydrates / Q000032:analysis; D002675:Child, Preschool; D002855:Chromatography, Thin Layer; D005260:Female; D005645:Fucosidosis / Q000378:metabolism*; D006801:Humans; D016256:Lewis X Antigen / Q000737:chemistry* / Q000302:isolation & purification; D008745:Methylation; D008969:Molecular Sequence Data; D010487:Periaqueductal Gray / Q000737:chemistry; D016339:Spectrometry, Mass, Fast Atom Bombardment
D002241:Carbohydrates; D016256:Lewis X Antigen