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Citations to this article

Metabolism of cerebroside sulfate and subcellular distribution of its metabolites in cultured skin fibroblasts from controls, metachromatic leukodystrophy, and globoid cell leukodystrophy.
K Inui, … , S Okada, H Yabuuchi
K Inui, … , S Okada, H Yabuuchi
Published February 1, 1988
Citation Information: J Clin Invest. 1988;81(2):310-317. https://doi.org/10.1172/JCI113322.
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Research Article

Metabolism of cerebroside sulfate and subcellular distribution of its metabolites in cultured skin fibroblasts from controls, metachromatic leukodystrophy, and globoid cell leukodystrophy.

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Abstract

With pulse-chase study of 1-[14C]stearic acid-labeled cerebroside sulfate (14C-CS) and subsequent subcellular fractionation by Percoll gradient, the metabolism of CS and translocation of its metabolites in human skin fibroblasts from controls, metachromatic leukodystrophy (MLD), and globoid cell leukodystrophy (GLD) were studied. In control skin fibroblasts, CS was transported to lysosome and metabolized there to galactosylceramide (GalCer) and ceramide (Cer) within 1 h. During the chase period, radioactivity was increased at plasma membrane plus Golgi as phospholipids and no accumulation of GalCer or Cer was found in lysosome. In MLD fibroblasts, 95% of 14C-CS taken up was unhydrolyzed at 24 h-chase and accumulated at not only lysosome but also plasma membrane. In GLD fibroblasts, GalCer was accumulated throughout the subcellular fractions and more accumulated mainly at plasma membrane plus Golgi with longer pulse. This translocation of lipid from lysosome seems to have considerable function, even in lipidosis, which may result in an imbalance of the sphingolipid pattern on the cell surface and these changes might be one of causes of neuronal dysfunction in sphingolipidosis.

Authors

K Inui, M Furukawa, S Okada, H Yabuuchi

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Total citations by year

Year: 2020 2005 2002 1996 1994 1992 1991 1990 Total
Citations: 1 1 1 1 1 1 2 3 11
Citation information
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Citations to this article in year 2005 (1)

Title and authors Publication Year
Magnetic Resonance of Myelination and Myelin Disorders
MS van der Knaap, J Valk
2005

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