✦ LIBER ✦

GM1-gangliosidosis: Accumulation of ganglioside GM1in cultured skin fibroblasts and correlation with clinical types

✍ Scribed by Yoshiyuki Suzuki; Norimasa Nakamura; Kazuko Fukuoka

Publisher: Springer
Year: 1978
Tongue: English
Weight: 270 KB
Volume: 43
Category: Article
ISSN: 0340-6717
DOI: 10.1007/bf00293589

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✦ Synopsis

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 types of this disease. The fibroblasts from an infantile-type patient showed a marked increase of GM1. In late-onset types the amount of total gangliosides was only slightly increased, but the distribution of individual gangliosides was definitely abnormal; a relative increase of GM1 was demonstrated in these cases. GM1 beta-galactosidase activities were not detectable in either infantile or late-onset cases.

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