Gangliosides in Human Skin Fibroblasts and their Enrichment in the “Hurler Variant” and Krabbe's Disease

Structure and quantities of gangliosides in skin fibroblasts derived from normal children and patients with several hereditary diseases are studied. Mono and disialohematosides are the only gangliosides found. There was a 6-fold increase in lipid-bound neuraminic acid in the Hurler variant and 2.3-fold increase in Krabbe's diesease as compared to the highest normal cells grown under the same conditions. The addition of calf-serum to the growth medium caused a substantial increase in the hematosides and also the occurrence of N-glycoloylneuraminic acid besides N-acetylneuraminic acid.