Purification and Properties of Two Sphingolipid Hydrolases

  • Howard R. Sloan
  • Jan L. Breslow
  • Donald S. Fredrickson
Part of the Advances in Experimental Medicine and Biology book series (AEMB, volume 19)


Our understanding of the lipid storage disorders has progressed dramatically since 1881 when Warren Tay, a British opthalmologist, described a cherry-red macular degeneration in the fundus of an infant with marked weakness of the trunk and limbs (1). This patient was the first reported case of Tay-Sachs disease. In the 90 years that have followed, there has been a delineation of a large number of inherited met abolic disorders characterized by the accumulation within tissues of large amounts of lipids.


Sodium Cholate Amniotic Fluid Cell Acetone Fraction Decyl Sulfate Sphingomyelinase Activity 
These keywords were added by machine and not by the authors. This process is experimental and the keywords may be updated as the learning algorithm improves.


Unable to display preview. Download preview PDF.

Unable to display preview. Download preview PDF.


  1. 1.
    Tay, W., Trans. Ophthal. Soc. U.K., 1, 155 (1881).Google Scholar
  2. 2.
    Weinreb, N. J., Brady, R. O. and Tappel, A. L., Biochim. Biophys. Acta 159, 141 (1968).CrossRefGoogle Scholar
  3. 3.
    Cuatrecasas, P., J. Biol. Chem. 245, 3059 (1970).PubMedGoogle Scholar
  4. 4.
    Carter, H. E. and Fujino, Y., J. Biol. Chem. 221, 879 (1956).PubMedGoogle Scholar
  5. 5.
    Kaller, H., Biochem. Zeitschrift 334, 451 (1961).Google Scholar
  6. 6.
    Chesebro, B. W., Personal Communication.Google Scholar
  7. 7.
    Breslow, J. L. and Sloan, H. R., in review.Google Scholar
  8. 8.
    Ornstein, L., Ann. N. Y. Acad. Sci. 121, 321 (1964).CrossRefGoogle Scholar
  9. 9.
    Davis, B. J., Ann. N. Y. Acad. Sci. 121, 404 (1964).CrossRefGoogle Scholar
  10. 10.
    Baum, H., Dodgson, K. S. and Spencer, B., Clin. Chim. Acta 4, 453 (1959).PubMedCrossRefGoogle Scholar
  11. 11.
    Sloan, H. R., Chem. Phys. Lipids 5, 250 (1970).PubMedCrossRefGoogle Scholar
  12. 12.
    Sloan, H. R., Uhlendorf, B. W., Kanger, J. N., Brady, R. O., and Fredrickson, D. S., Biochem. Biophys. Res. Commun. 34, 582 (1969).CrossRefGoogle Scholar
  13. 13.
    Kwiterovich, P. O., Sloan, H. R. and Fredrickson, D. S., J. Lipid Res. 11, 322 (1970).PubMedGoogle Scholar
  14. 14.
    Mapes, C. A., Anderson, R. L., Sweeley, C. C., Desnick, R. J. and Krivit, W., Science 169, 987 (1970).PubMedCrossRefGoogle Scholar
  15. 15.
    Brady, R. O., Bulletin of the New York Academy of Medicine 47, 173 (1971).PubMedGoogle Scholar

Copyright information

© Springer Science+Business Media New York 1972

Authors and Affiliations

  • Howard R. Sloan
    • 1
  • Jan L. Breslow
    • 1
  • Donald S. Fredrickson
    • 1
  1. 1.Molecular Disease BranchNational Heart and Lung InstituteUSA

Personalised recommendations