Results 61 to 70 of about 565 (80)
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Biochemical and Biophysical Research Communications, 1971
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 ...
Arvan L Fluharty, Hayato Kihara
exaly +3 more sources
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 ...
Arvan L Fluharty, Hayato Kihara
exaly +3 more sources
Late-onset Krabbe disease initially diagnosed as cerebroside sulfatase activator deficiency
Metabolic Brain Disease, 1986Clinical and biochemical findings in a male subject with progressive encephalopathy and peripheral neuropathy are presented. Early development was normal. At age 3.5 years, he had seizures associated with fever. Subsequently, there was progressive neurologic deterioration. A CT brain scan at age 4 years, 2 months demonstrated multiple areas of variable
Arvan L Fluharty +2 more
exaly +3 more sources
Lipids and Lipid Metabolism, 1990
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 ...
Lambert M G Van Golde +2 more
exaly +3 more sources
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 ...
Lambert M G Van Golde +2 more
exaly +3 more sources
Analytical Biochemistry, 1980
Abstract A convenient method for large scale preparation of stearoyl[1- 14 C]sulfogalactosylsphingosine has been developed. The first step consists in preparing the lysosulfatide intermediate with a good yield, which we have successfully performed. In the second step, the lysoderivative is coupled to [1- 14 C]stearic acid through the acyl chloride ...
G Dubois, B Zalc, F Le Saux
exaly +3 more sources
Abstract A convenient method for large scale preparation of stearoyl[1- 14 C]sulfogalactosylsphingosine has been developed. The first step consists in preparing the lysosulfatide intermediate with a good yield, which we have successfully performed. In the second step, the lysoderivative is coupled to [1- 14 C]stearic acid through the acyl chloride ...
G Dubois, B Zalc, F Le Saux
exaly +3 more sources
Cerebroside sulfatase activity in cultivated human skin fibroblasts and amniotic fluid cells
Journal of Pediatrics, 1975In 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 ...
Henry L Nadler
exaly +3 more sources
Human Genetics, 1984
Several cases of metachromatic leukodystrophy (MLD) have been described with normal or near normal activities of arylsulfatase A (cerebroside sulfatase). However, the ability of intact cultured fibroblasts to hydrolyze cerebroside sulfate was impaired. Since the impairment was corrected by cerebroside sulfatase activator, a deficiency of activator was ...
Arvan L Fluharty
exaly +3 more sources
Several cases of metachromatic leukodystrophy (MLD) have been described with normal or near normal activities of arylsulfatase A (cerebroside sulfatase). However, the ability of intact cultured fibroblasts to hydrolyze cerebroside sulfate was impaired. Since the impairment was corrected by cerebroside sulfatase activator, a deficiency of activator was ...
Arvan L Fluharty
exaly +3 more sources
CEREBROSIDE SULFATASE ACTIVATOR DEFICIENCY
1984Arvan L Fluharty, Hayato Kihara
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Discussion: Metachromatic Leukodystrophy, An Unusual Case with a Subtle Cerebroside Sulfatase Defect
1975Hayato Kihara
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The Specificity of Cerebroside Sulfatase Activator
1986The unability for the lysosomal lipid hydrolases to act on their natural substrate alone, has been reported a long time ago (1). They showed the necessity to add a “detergent-like” natural protein called activator protein which allows the hydrolysis of the sulfatides by cerebroside sulfatase.
Gisèle Dubois +2 more
openaire +1 more source