Abstract
Detailed chemical analyses of the acid mucopolysaccharides of liver, brain and urine in multiple sulphatase deficiency have shown heparin sulphate to be the major component.
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Austin, J., Armstrong, D. and Shearer, L. Metachromatic form of diffuse cerebral sclerosis (metachromatic leukodystrophy; MLD). V. The nature and significance of low sulfatase activity: a control of low sulfatase activity; a control study of brain, liver and kidney in four patients with MLD.Arch. Neurol. 13 (1965) 593–614
Brunngraber, E. G., Brown, B. D. and Aguilar, V. Isolation and determination of non-diffusible sialofucohexoaminoglycans derived from brain glycoproteins and their anatomical distribution in bovine brain.J. Neurochem. 16 (1969) 1059–1070
DiFerrante, N. Acid mucopolysaccharides of normal human urine.J. Lab. Clin. Med. 61 (1963) 633–641
Eto, Y., Wiesmann, U. N., Carson, J. H. and Herschkowitz, N. N. Multiple sulfatase deficiency in cultured skin fibroblasts; occurrence in patients with a variant form of metachromatic leukodystrophy.Arch. Neurol. 30 (1974) 153–157
Fransson, L. A. and Rodén, L. J. Structure of dermatan sulfate. I. Degradation of testicular hyaluronidase.J. Biol. Chem. 242 (1967) 4161–4169
Murata, K. Acidic glycosaminoglycans in human kidney tissue.Clin. Chim. Acta 63 (1975) 157–169
Murata, K., Harada, T. and Fujiwara, T. Chondroitin sulfate isomers in normal human urine.Biochim. Biophys. Acta 230 (1971) 583–594
Murphy, J. V., Wolfe, J. V., Wolfe, H. J., Balazs, E. A. and Moser, H. W. A patient with deficiency of arylsulfatase A, B, C and steroid sulphatases, associated with storage of sulfatide, cholesterol sulfate and glycosaminoglycans. In Bernsohn, J. and Grossman, H. J. (eds.)Lipid Storage Disorders; Enzymatic Defects and Clinical Implications, Academic Press, New York, 1971, pp. 67–101
Schiller, S., Slover, G. A. and Dorfman, A. A method for the separation of acid mucopolysaccharides. Its application to the isolation of heparin from the skin of rats.J. Biol. Chem. 236 (1961) 983–988
Tokunaga, M., Wakamatsu, E. and Yoshizawa, Z. Increased excretion of urinary glycosaminoglycans in a case of Rothmund Thompson syndrome.Tohuku J. Exp. Med. 126 (1978) 1–6
Wessler, E. Analytical and preparative separation of acidic glycosaminoglycans by electrophoresis in barium acetate.Anal. Biochem. 26 (1968) 439–444
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Eto, Y., Tokoro, T., Ito, F. et al. Chemical compositions of acid mucopolysaccharides in urine and tissues of patients with multiple sulphatase deficiency. J Inherit Metab Dis 4, 161–162 (1981). https://doi.org/10.1007/BF02263640
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DOI: https://doi.org/10.1007/BF02263640