Accumulation of lactosyl ceramide in leukocytes of patients with adult Gaucher's disease.
Glycosphingolipids were isolated from the leukocytes of 10 patients with Type I, chronic nonneuronopathic (adult) Gaucher's disease and 12 normal subjects, by silicic acid column chromatography and thin-layer chromatography. Quantitation of the individual glycosyl ceramides was achieved by the determination of hexose and sphingosine content, using colorimetric and fluorometric procedures. Lactosyl ceramide, which is the main glycolipid in leukocytes of normal subjects, was significantly increased in the leukocytes of patients with Gaucher's disease. On the other hand, the amount of glycosyl ceramide, which is the main glycolipid accumulating in the reticuloendothelial cells of patients with Gaucher's disease, was similar in Gaucher and in control leukocytes.
D000328:Adult; D000368:Aged; D002554:Cerebrosides / Q000097:blood*; D002648:Child; D005260:Female; D005690:Galactose / Q000032:analysis; D005776:Gaucher Disease / Q000097:blood*; D005947:Glucose / Q000032:analysis; D006028:Glycosphingolipids / Q000097:blood*; D006801:Humans; D007785:Lactose / Q000031:analogs & derivatives / Q000032:analysis / Q000097:blood; D007962:Leukocytes / Q000378:metabolism*; D008297:Male; D008875:Middle Aged
D002554:Cerebrosides; D006028:Glycosphingolipids; D005947:Glucose; D007785:Lactose; D005690:Galactose