Results 101 to 110 of about 620 (115)
Summary Despite the absence of cerebroside sulfatase activity in cellfree preparations, fibroblasts in culture derived from patients with metachromatic leukodystrophy were capable of hydrolyzing exogenous cerebroside sulfate. Moreover, the degree of whole-cell sulfatase activity was directly correlated to the age of onset of clinical symptoms in the ...
M T, Porter +3 more
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CEREBROSIDE SULFATASE ACTIVATOR DEFICIENCY
Arvan L. Fluharty, Hayato Kihara
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Discussion: Metachromatic Leukodystrophy, An Unusual Case with a Subtle Cerebroside Sulfatase Defect
Hayato Kihara
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The in vivo metabolism of sulfatides was studied in spinal cord and cerebral cortex of developing rat pups. Developmental changes in the rate of sulfolipid synthesis were measured after the intraperitoneal injection of 35SO4(2-). We also measured the accumulation of sulfatides, as well as the profiles of cerebroside sulfotransferase, cerebroside ...
R H, van der Pal +3 more
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Inhibition of specific opiate binding to synaptic membrane by cerebroside sulfatase
Abstract The role of cerebroside sulfate in the specific binding of opiates to the synaptic membrane was investigated by the use of the enzyme, sulfatase A (cerebroside sulfatase, cerebroside-3-sulfate-3-sulphohydrolyase, EC 3.1.6.8). It was observed that the hydrolysis of sulfatides in the synaptic membrane by this enzyme was dependent on the ...
P Y, Law, G, Fischer, H H, Loh, A, Herz
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Cerebroside sulfatase activity in cultivated human skin fibroblasts and amniotic fluid cells
In an effort to improve the precision in prenatal monitoring for metachromatic leukodystrophy, levels of cerebroside sulfatase were determined in fibroblasts and amniotic fluid cells. Cells from MLD patients demonstrated no significant sulfatide hydrolysis, whereas cultures from heterozygous subjects hydrolyzed diminished but definite amounts of ...
C W, Booth, K K, Chen, H L, Nadler
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Cerebroside sulfatase activator deficiency induced metachromatic leukodystrophy.
American journal of human genetics, 1982Two siblings of consanguineous parents had presented with a variety of findings indicative of juvenile metachromatic leukodystrophy (MLD). However, instead of the expected profound deficiency of arylsulfatase A (ARS A), their enzyme levels were about half-normal, and enzyme from fibroblasts had properties identical with the properties of enzyme from ...
R L, Stevens +7 more
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[Purification and characterization of cerebroside sulfatase activator].
[Hokkaido igaku zasshi] The Hokkaido journal of medical science, 1984Cerebroside sulfatase (CSase) activator was isolated from human liver by acetone precipitation, anion-exchange chromatography, gel filtration and polyacrylamide gel electrophoresis. The CSase activator was a heat-stable protein with an isoelectric point of 4.54.
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