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E. Mehl (1963)
Über ein Cerebrosidschwefelsäureester Spaltendes Enzym aus SchweineniereHoppe-Seyler's Z Physiol Chem, 331
E. Mehl, H. Jatzkewitz (1963)
[On an enzyme from swine kidneys which cleave cerebroside sulfuric acid esters].Hoppe-Seyler's Zeitschrift fur physiologische Chemie, 331
O. Lowry, N. Rosebrough, A. Farr, R. Randall (1951)
Protein measurement with the Folin phenol reagent.The Journal of biological chemistry, 193 1
K. Dodgson (1957)
Studies on SulphatasesBiochem J, 65
A. Roy (1963)
Die Wirkung der Arylsulfatasen A und B auf CholesterinsulfatHoppe-Seyler's Zeit für Physiol Chem, 333
S. Suzuki , N. Takahashi, F. and Egami (1957)
Enzymic Transsulfation From Phenol to CarbohydratesBiochem Biophys Acta, 24
A. deReuck (1963)
Lysosomes
Y. Fujino, T. Negishi (1952)
Studies on the Conjugated LipidsBulletin of the Agricultural Chemical Society of Japan, 24
J. Austin (1960)
Metachromatic Form of Diffuse Cerebral Sclerosis: III. Significance of Sulfatide and Other Lipid Abnormalities in White Matter and KidneyNeurology, 10
J. Austin, A. Balasubramanian, T. Pattabiraman, S. Saraswathi, D. Basu, B. Bachhawat (1963)
A CONTROLLED STUDY OF ENZYMIC ACTIVITIES IN THREE HUMAN DISORDERS OF GLYCOLIPID METABOLISM *Journal of Neurochemistry, 10
J. Austin (1960)
Metachromatic form of diffuse cerebral sclerosisNeurology, 10
S. Suzuki, N. Takahashi, F. Egami (1957)
Enzymic transsulfation from a phenol to carbohydrates.Biochimica et biophysica acta, 24 2
H. Baum (1959)
Dodgson, K.Clin Chim Acta, 4
K. Dodgson, B. Spencer (1957)
Studies on sulphatases. 15. The arylsulphatases of human serum and urineBiochemical Journal, 65
Y. Fujino (1957)
Studies on Conjugated LipidsBull Agr Chem Soc Japan, 21
James Austin, Margaret Bischel (1961)
A histochemical method for sulfatase activity in hemic cells and organ imprints.Blood, 17
A. Roy (1963)
[THE EFFECT OF ARYLSULFATASES A AND B ON CHOLESTEROL SULFATE].Hoppe-Seyler's Zeitschrift fur physiologische Chemie, 333
E. Boyland, D. Wallace, D. Williams (1955)
The Activity of the Enzymes Sulphatase and β-Glucuronidase in the Urine, Serum and Bladder TissueBritish Journal of Cancer, 9
E. Mehl (1964)
Proceedings of the Sixth International Congress on Biochemistry, 4
J.: " Austin (1964)
Medical Aspects of Mental Retardation
A. Roy (1954)
The sulphatase of ox liver. 3. Further observations on sulphatase B and an investigation of the origin of fractions A and B.The Biochemical journal, 57 3
A. Roy (1958)
Comparative studies on the liver sulphatases.The Biochemical journal, 68 3
J. Austin (1958)
Observations in Metachromatic LeucoencephalopathyTrans Amer Neurol Assoc
J. Austin (1957)
Metachromatic Form of Diffuse Cerebral Sclerosis: I. Diagnosis During Life by Urine Sediment ExaminationNeurology, 7
J. Austin
Low Sulphatase Activities in Metachromatic Leukodystrophy (MLD): Controlled Study of Enzymes in Nine Living and in Four Autopsied Patients with MLDTrans Amer Neurol Ass
Abstract A STATISTICALLY significant decrease in sulfatase activity has been noted in autopsy tissues from four patients with metachromatic leukodystrophy (MLD) (sulfatide lipidosis).1,19 Arylsulfatase activity was low both in histochemical tests2,14 and in quantitative colorimetric analyses of brain, liver, and kidney. It would seem reasonable to relate a sulfatase deficiency in some way to the excess of cerebroside sulfates (sulfatides) found in the central nervous system and kidney in MLD.5 Enzyme studies on dead patients are limited by several important qualifications. These restrict the conclusions which can legitimately be drawn from postmortem studies alone even when the analyses are well controlled. Hence, the present study was designed to test a critical question—is sulfatase activity low in living MLD patients who are still in earlier stages of their disorder? Urines were selected for assay because sulfatase activity which is produced in the kidney normally enters the urine in detectable References 1. Austin, J., et al. Controlled Study of Enzymic Activities in Three Human Disorders of Glycolipid Metabolism , J Neurochem 10:805-816, 1963.Crossref 2. Austin, J.: Unpublished data. 3. Austin, J.: Metachromatic Form of Diffuse Cerebral Sclerosis: I. Diagnosis During Life by Urine Sediment Examination , Neurology 7:415-426, 1957.Crossref 4. Austin, J.: Observations in Metachromatic Leucoencephalopathy , Trans Amer Neurol Assoc 1958, pp 149-152. 5. Austin, J.: Metachromatic Form of Diffuse Cerebral Sclerosis: III. Significance of Sulfatide and Other Lipid Abnormalities in White Matter and Kidney , Neurology 10:470-483, 1960.Crossref 6. Austin, J.: " Metachromatic Leukodystrophy ," in Medical Aspects of Mental Retardation , Springfield, Ill: Charles C Thomas, Publisher, 1964. 7. Roy, A.: Sulphatase of Ox Liver: III. Further Observations on Sulphatase B and Investigation of Origin of Fractions A and B , Biochem J 57:465-470, 1954. 8. Mehl, E., and Jatzkewitz, H.: Über ein Cerebrosidschwefelsäureester Spaltendes Enzym aus Schweineniere , Hoppe-Seyler's Z Physiol Chem 331:292-294, 1963.Crossref 9. Roy, A.: Comparative Studies on Liver Sulphatases , Biochem J 68:519-528, 1958. 10. Baum, H.: Dodgson, K.; and Spencer, B.: Assay of Arylsulphatases A and B in Human Urine , Clin Chim Acta 4:453-455, 1959.Crossref 11. Lowry, O., et al: Protein Measurement With Folin Phenol Reagent , J Biol Chem 193:265-275, 1951. 12. Dodgson, K., and Spencer, B.: Studies on Sulphatases , Biochem J 65:668-673, 1957. 13. Mehl, E., and Jatzketwitz, H.: Mammalian Cerebroside Sulfatase and Its Function , in Proceedings of the Sixth International Congress on Biochemistry , 4:108, 1964. 14. Austin, J., and Bischel, M.: Histochemical Method for Sulfatase Activity in Hemic Cells and Organ Imprints , Blood 17:216-224, 1961. 15. Boyland, E.; Wallace, D.; and Williams, D.: Activity of Enzymes Sulphatase and β-Glucuronidase in Urine, Serum, and Bladder Tissue , Brit J Cancer 9:62-77, 1955.Crossref 16. Sigma Chemical Co., technical bulletin No. 104, St. Louis, Mo, revised March 1963. 17. Suzuki, S.; Takahashi, N.; and Egami, F.: Enzymic Transsulfation From Phenol to Carbohydrates , Biochem Biophys Acta 24:444-445, 1957.Crossref 18. Fujino, Y., and Negishi, T.: Studies on Conjugated Lipids , Bull Agr Chem Soc Japan 21:225-229, 1957.Crossref 19. Austin, J., et al: Low Sulphatase Activities in Metachromatic Leukodystrophy (MLD): Controlled Study of Enzymes in Nine Living and in Four Autopsied Patients with MLD , Trans Amer Neurol Ass , to be published. 20. deReuck, A., and Cameron, M., Eds.: Lysosomes , Boston: Little Brown and Co., 1963, pp 1-446. 21. Roy, A.: Die Wirkung der Arylsulfatasen A und B auf Cholesterinsulfat , Hoppe-Seyler's Zeit für Physiol Chem 333:166-168, 1963.Crossref 22. Austin, J., et al: Metachromatic Form of Diffuse Cerebral Sclerosis (Metachromatic Leukodystrophy; MLD): V. Low Sulphatase Activity in Controlled Study of Brain, Liver, and Kidney of Four MLD Patients. In preparation for submission to this journal. 23. Austin, J., et al: Metachromatic Form of Diffuse Cerebral Sclerosis (Metachromatic Leukodystrophy; MLD): VI. Rapid Clinical Laboratory Test for Sulphatase Deficiency. In preparation for submission to this journal.
Archives of Neurology – American Medical Association
Published: May 1, 1965
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