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
- 1 September 1987
- journal article
- conference paper
- Published by Wiley in Journal of Inherited Metabolic Disease
- Vol. 10 (3) , 293-296
- https://doi.org/10.1007/bf01800085
Abstract
No abstract availableKeywords
This publication has 8 references indexed in Scilit:
- Hexosaminidase a activity in skin fibroblasts from various types of GM2 gangliosidosis using a fluorogenic sulphated substrateJournal of Inherited Metabolic Disease, 1985
- Fluorescence Polarization Analysis, Lipid Composition, and Na+, K+‐ATPase Kinetics of Synaptosomal Membranes in Feline GM1 and GM2 GangliosidosisJournal of Neurochemistry, 1985
- Intracellular translocation of fluorescent sphingolipids in cultured fibroblasts: endogenously synthesized sphingomyelin and glucocerebroside analogues pass through the Golgi apparatus en route to the plasma membrane.The Journal of cell biology, 1985
- JUVENILE GM2 GANGLIOSIDOSIS (AMB VARIANT) - INABILITY TO ACTIVATE HEXOSAMINIDASE-A BY ACTIVATOR PROTEIN1983
- Diagnosis of Metachromatic Leukodystrophy, Krabbe Disease, and Farber Disease after Uptake of Fatty Acid-labeled Cerebroside Sulfate into Cultured Skin FibroblastsJournal of Clinical Investigation, 1982
- Stearoyl[1-14C]sulfogalactosylsphingosine ([14C]sulfatide) as substrate for cerebroside sulfatase assayAnalytical Biochemistry, 1980
- Cerebroside sulfate hydrolysis by fibroblasts from a parent with metachromatic leukodystrophyThe Journal of Pediatrics, 1978
- Distortion of neuronal geometry and formation of aberrant synapses in neuronal storage diseaseBrain Research, 1976