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Lactosylceramidosis: Lactosylceramide galactosyl hydrolase deficiency and accumulation of lactosylceramide in cultured skin fibroblasts

โœ Scribed by Glyn Dawson; Reuben Matalon; Arthur O. Stein


Book ID
118536494
Publisher
Elsevier Science
Year
1971
Tongue
English
Weight
843 KB
Volume
79
Category
Article
ISSN
1097-6833

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โœ Yoshiyuki Suzuki; Norimasa Nakamura; Kazuko Fukuoka ๐Ÿ“‚ Article ๐Ÿ“… 1978 ๐Ÿ› Springer ๐ŸŒ English โš– 270 KB

Uptake of radioactivity from 14C-galactose into gangliosides by cultured skin fibroblasts was studied. GM3 was the major ganglioside in control human fibroblasts. An increase of GM1 was demonstrated in GM1-gangliosidosis fibroblasts. The degree of GM1 accumulation was correlated with the clinical ty