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Metachromatic Form of Diffuse Cerebral Sclerosis: IV. Low Sulfatase Activity In the Urine Of Nine Living Patients With Metachromatic Lcukodystrophy (MLD)

Metachromatic Form of Diffuse Cerebral Sclerosis: IV. Low Sulfatase Activity In the Urine Of Nine... 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 Austin, J.: Unpublished data. 3.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. http://www.deepdyve.com/assets/images/DeepDyve-Logo-lg.png Archives of Neurology American Medical Association

Metachromatic Form of Diffuse Cerebral Sclerosis: IV. Low Sulfatase Activity In the Urine Of Nine Living Patients With Metachromatic Lcukodystrophy (MLD)

Archives of Neurology , Volume 12 (5) – May 1, 1965

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References (25)

Publisher
American Medical Association
Copyright
Copyright © 1965 American Medical Association. All Rights Reserved.
ISSN
0003-9942
eISSN
1538-3687
DOI
10.1001/archneur.1965.00460290003001
Publisher site
See Article on Publisher Site

Abstract

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.

Journal

Archives of NeurologyAmerican Medical Association

Published: May 1, 1965

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