Vitamin C-Induced Increase of Dermatan Sulfate in Cultured Hurler's Fibroblasts

Schafer, Irwin A.; Sullivan, Julia C.; Kofoed, J. A.; Robertson, W. W.

doi:10.1126/science.153.3739.1008

Cited by 26 publications

(14 citation statements)

References 8 publications

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“…The data reported here extend and amplify preliminary studies from this laboratory which demonstrated that the classically accepted biochemical phenotype of Hurler syndrome, i.e., increased dermatan sulfate concentration within cells, persisted in fibroblast cultures derived from the skin of Hurler patients (10). Additional data have been obtained indicating that the biochemical defect is not limited to increased sulfated GAG synthesis but is also characterized by increased synthesis of nonsulfated GAG.…”

Section: The Journal Of Clinical Investigation Volume 47 1968supporting

confidence: 73%

Study of the Hurler syndrome using cell culture: definition of the biochemical phenotype and the effect of ascorbic acid on the mutant cell

Schafer¹,

Sullivan²,

Kofoed³

et al. 1968

J. Clin. Invest.

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Section: The Journal Of Clinical Investigation Volume 47 1968supporting

confidence: 73%

Study of the Hurler syndrome using cell culture: definition of the biochemical phenotype and the effect of ascorbic acid on the mutant cell

Schafer¹,

Sullivan²,

Kofoed³

et al. 1968

J. Clin. Invest.

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“…An increased accumulation of dermatan sulfate was found in the cell layer of Hurler syndrome fibroblasts when ascorbic acid was added to the culture medium (33). In scorbutic guinea pigs, cartilage cells were found to contain reduced levels of glycosaminoglycans, DNA, and glycogen.…”

Section: Resultsmentioning

confidence: 99%

Effect of ascorbic acid on arylsulfatase activities and sulfated proteoglycan metabolism in chondrocyte cultures.

Schwartz¹,

Adamy²

1977

J. Clin. Invest.

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A B S T R A C T A correlation between increased arylsulfatase activities and decreased sulfated proteoglycan content in human osteoarthritic articular cartilage suggested a possible interrelationship between these parameters. Since we had previously shown that ascorbate caused a decrease in levels of arylsulfatase A and B activities in normal chondrocyte cultures, the validity of the above relationship was examined by measuring the effect of vitamin C on the biosynthesis and distribution of 35S-labeled proteoglyeans and arylsulfatase A and B activities in cell extracts of chondrocytes derived from normal and osteoarthritic tissue.Arylsulfatase A and B activities were found to be reduced in the presence of ascorbic acid in all normal and osteoarthritic cell lines examined when measured 3, 6, 10, and 13 days after the introduction of the vitamin in the culture medium. Acid phosphatase activity, on the other hand, was found to be elevated in the presence of ascorbate.The inhibitory effect by ascorbic acid on arylsulfatase activities could be reversed by withdrawing the vitamin from the nutrient medium. Addition of EDTA to the cell extracts before assay also reversed the inhibition.Sulfated proteoglycan biosynthesis as reflected in 35S-sulfate uptake per milligram of DNA was significantly increased in the presence of ascorbic acid. The distribution of the newly synthesized molecules between the cell layer and medium fractions was altered. In the presence of ascorbate, more deposition into the cell layer of newly synthesized macromolecules occurred.These data suggest an inverse relationship between arylsulfatase activities and the stability of the newly synthesized sulfated proteoglycans in the extracellular matrix.

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“…The observation that vitamin C increases the synthesis of glycosaminoglycans by skin fibroblasts (Schafer, Sullivan, Svejcar, Kofoed, and Robertson, 1966) whereas retinol reduces it (Danes and Beam, 1966b) offers a possible approach to therapy, though the basic defect is unlikely to be modified in this way.…”

Section: Resultsmentioning

confidence: 99%

Urinary excretion of glycosaminoglycans in the various forms of gargoylism

Manley

Williams

1969

J Clin Pathol

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The urinary excretion of glycosaminoglycans in 28 cases of gargoylism, embracing the Hurler, Hunter, Sanfilippo, Morquio, and Scheie syndromes (McKusick, 1966), has been examined using the cetylpyridinium chloride (CPC) turbidity test, the uronic acid/creatinine ratio, and the electrophoretic pattern of urine concentrates, as routine procedures. Ion-exchange column chromatographic techniques were also employed for the fractionation of glycosaminoglycans and aminosugars. Molecular weights were investigated by gel filtration and ultracentrifugation.The CPC turbidity test was positive in every case. The uronic acid/creatinine ratio provided a sensitive index of increased glycosaminoglycan excretion. Cases of the Hurler syndrome showed the highest, and cases of the Morquio and Scheie syndromes the lowest, ratios. A correlation was observed between the uronic acid/creatinine ratio and the clinical severity of the disease. Cellulose acetate electrophoresis differentiated clearly between the two major forms of gargoylism, the Hurler and Sanfilippo syndromes, but differentiation between the Hurler, Hunter, and Scheie syndromes was more difficult on electrophoretic data alone. Results obtained with cases diagnosed as the Morquio syndrome were disappointing. The existence offormes frustesof the Sanfilippo syndrome among the mentally subnormal is predicted. Errors caused by bacterial contamination of urine samples are emphasized. The atypical behaviour of urinary glycosaminoglycans in analytical procedures is discussed. Molecular weight studies suggested heterogeneity. The nature of the basic defect in gargoylism is discussed.

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Vitamin C-Induced Increase of Dermatan Sulfate in Cultured Hurler's Fibroblasts

Cited by 26 publications

References 8 publications

Study of the Hurler syndrome using cell culture: definition of the biochemical phenotype and the effect of ascorbic acid on the mutant cell

Study of the Hurler syndrome using cell culture: definition of the biochemical phenotype and the effect of ascorbic acid on the mutant cell

Effect of ascorbic acid on arylsulfatase activities and sulfated proteoglycan metabolism in chondrocyte cultures.

Urinary excretion of glycosaminoglycans in the various forms of gargoylism

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