References
Dobois, G., Zak, B., Le Saux, F. and Baumann, N. Stearoyl (1-14C) sulfogalactosylsphingosine ((14C)sulfatide) as substrate for cerebroside sulfatase assay.Anal. Chem. 102 (1980) 313–317
Fluharty, A. L., Stevens, R. L. and Kihara, H. Cerebroside sulfate hydrolysis by fibroblasts from a patient with metachromatic leukodystrophy.J. Pediatr. 92 (1978) 782–784
Inui, K., Grebner, E. E., Jackson, L. G. and Wenger, D. A. Juvenile GM2 Gangliosidosis (AMB Variant): Inability to activate hexosaminidase A by activator protein.Am. J. Hum. Genet. 35 (1983) 551–564
Inui, K., Okada, S., Yabuuchi, H., Wenger, D. A. and Desnick, R. J. Hexosaminidase A activity in skin fibroblasts from various types of GM2 gangliosidosis using a fluorogenic sulphated substrate.J. Inher. Metab. Dis. 8 (1985) 149–150
Kudoh, T. and Wenger, D. A. Diagnosis of metachromatic leukodystrophy, Krabbe disease, and Farber disease after uptake of fatty acid-labelled cerebroside sulfate into cultured skin fibroblasts.J. Clin. Invest. 70 (1982) 89–97
Lipsky, N. G. and Pagono, R. E. Intercellular translocation of fluorescent sphingolipids in cultured fibroblasts: Endogenously synthesized sphingomyelin and glucocerebroside analogues pass through the Golgi apparatus en route to the plasma membrane.J. Cell Biol. 100 (1985) 27–34
Purpura, D. P. and Suzuki, K. Distortion of neuronal geometry and formation of abberant synapses in neuronal storage disease.Brain Res. 116 (1976) 1–21
Wood, P. A., McBride, M. A., Baker, H. J. and Christian, S. T. Fluorescence polarization analysis, lipid composition and Na+, K+-ATPase kinetics of synaptosomal membranes in feline GM1 and GM2 gangliosidosis.J. Neurochem. 44 (1985) 947–956
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Inui, K., Furukawa, M., Nishimoto, J. et al. Metabolism of cerebroside sulphate and subcellular distribution of its metabolites in cultured skin fibroblasts derived from controls, metachromatic leukodystrophy, globoid cell leukodystrophy and farber disease. J Inherit Metab Dis 10, 293–296 (1987). https://doi.org/10.1007/BF01800085
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DOI: https://doi.org/10.1007/BF01800085