Abstract
It is concluded that the defect in Tay-Sachs disease is for an enzyme degrading a monosialoganglioside, in Niemann-Pick disease for an enzyme degrading sphingomyelin or possibly ceramide, in chronic Gaucher’s disease for an enzyme degrading a glucocerebroside, and in metachromatic leucodystrophy for an enzyme for degradation of sulfatide. Alzheimer’s disease does not appear to involve any specific changes in lipid composition. An hypothesis to explain the findings in Alzheimer’s disease is presented.
Similar content being viewed by others
References
Frederickson, D. S., in “The Metabolic Basis Of Inherited Disease,” J. B. Stansbury, J. B. Wyngaarden, and D. S. Frederickson, Editors, McGraw-Hill Book Co., Inc., New York, 1960, pp. 553–636.
O’Brien, J. S., and G. Rouser, Anal. Bichem.7, 228–296 (1964).
Klenk, E., Z. physiol. Chem.262, 128–143 (1939–40).
Klenk, E., Z. physiol. Chem.235, 24–36 (1935).
Lieb, H., Z. physiol. Chem.140, 305–313 (1924).
Jatzkewitz, H., Z. physiol. Chem.,320, 134–148 (1960).
Rouser, G., C. Galli and G. Kritchevsky, JAOCS42, 404–410 (1965.)
Jatzkewitz, H., in Brain Lipids, Lipoproteins, Leucodystrophies, Proc. Neurochem. Symp., Rome, 1961, 147–152 (1963).
Rouser, G., A. J. Bauman, N. Nicolaides and D. Heller, JAOCS38, 565–581 (1961).
Booth, D. A., Biochim, Biophys. Acta70, 486–487 (1963).
Svennerholm, L., and A. Raal, Biochim. Biophys. Acta53, 422 (1961).
Svennerholm, L., in Brain Lipids, Lipoproteins, Leucodystrophies, Proc. Neurochem. Symp., Rome, 1961, 104–119 (1963).
O’Brien, J. S., Biochem. Biophys. Res. Commun.15, 484–490 (1964).
Berry, J. F., W. H. Cevallos and R. R. Wade, Jr., JAOCS (this Symposium).
Jatzkewitz, H., and E. Mehl, Z. physiol. Chem.329, 264–277 (1962).
Gatt, S., and E. R. Berman, J. Neurochem.10, 43–49 (1963).
Makita, A., and T. Yamakawa, Japan J. Exp. Med.33, 361–368 (1963).
Makita, A., and T. Yamakawa, J. Biochem.55, 365–370 (1964).
Rouser, G., G. Feldman, and C. Galli, JAOCS42, 411–412 (1965).
Author information
Authors and Affiliations
About this article
Cite this article
Rouser, G., Kritchevsky, G. & Galli, C. Speculations on the nature of the metabolic defects in Tay-Sachs, Niemann-Pick, Gaucher’s and Alzheimer’s diseases, and metachromatic leucodystrophy. J Am Oil Chem Soc 42, 412–416 (1965). https://doi.org/10.1007/BF02635578
Issue Date:
DOI: https://doi.org/10.1007/BF02635578