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Hydrolases of the Simple Sphingolipids: Ceramide, Glucocerebroside, and Galactocerebroside

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Abstract

Publications covering the sphingolipids in some depth are beginning to appear. Burton and Guerra (1974) have just edited a book on lipid chemistry which contains a detailed chapter by Burton on sphingolipids. Proceedings of a 1971 symposium contain useful informal reviews (Volk and Aronson, 1972). Another recent symposium collection by Bernsohn and Grossman (1971) contains articles on the hydrolases. A detailed review of sphingolipid metabolism by Morell and Braun (1972) covers the lipids lacking sialic acid. Gatt (1973) has one chapter on sphingolipid enzymes in a Milan symposium and one with Barenholz (1973) on enzymes of complex lipid metabolism in Annual Reviews. Chapters by Radin and Rosenberg (1970) on cerebrosides and sphingomyelin appeared in Volume 3 of Handbook of Neurochemistry. A chapter on glycolipid metabolism by Dawson (1974) has recently appeared in an edited volume.

Keywords

  • Bile Salt
  • Galactose Oxidase
  • Gauche Patient
  • Galactosyl Ceramide
  • Glucosyl Ceramide

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.

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References

  • Agranoff, B. W., Radin, N. S., and Suomi, W., 1962, Enzymatic oxidation of cerebrosides: studies on Gaucher’s disease, Biochim. Biophys. Acta 57: 194–196.

    PubMed  CAS  CrossRef  Google Scholar 

  • Anzil, A. P., Blinzinger, K., Mehraein, P., Dorn, G., and Neuhäuser, G., 1972, Cytoplasmic inclusions in a child afflicted with Krabbe’s disease (globoid leucodystrophy) and in the rabbit injected with galactocerebrosides, J. Neuropath. Exp. Neurol. 31: 370–388.

    PubMed  CAS  CrossRef  Google Scholar 

  • Arora, R. C., and Radin, N. S., 1972a, Ceramide-like synthetic amides that inhibit cerebroside galactosidase, J. Lipid Res. 13: 86–91.

    PubMed  CAS  Google Scholar 

  • Arora, R. C, and Radin, N. S., 19726, Stimulation in vitro of galactocerebroside galactosidase by N-decanoyl 2-amino-2-methylpropanol, Lipids 7: 56–59.

    Google Scholar 

  • Arora, R. C, Lin, Y.-N., and Radin, N. S., 1973, The inhibitor-sensitive sites of galactosyl ceramide galactosidase, Arch. Biochem. Biophys. 156: 77–83.

    PubMed  CAS  CrossRef  Google Scholar 

  • Austin, J. H., and Lehfeldt, D., 1965, Studies in globoid (Krabbe) leukodystrophy. 3. Significance of experimentally produced globoidlike elements in rat white matter and spleen, J. Neuropathol. Exptl. Neurol. 24: 265–289.

    CAS  CrossRef  Google Scholar 

  • Benjamins, J. A., Fitch, J., and Radin, N. S., Effects of ceramide analogs on myelinating organ cultures. Submitted for publication.

    Google Scholar 

  • Bergsma, D., 1973, Enzyme Therapy in Genetic Diseases, The Williams and Wilkins Co., Baltimore.

    Google Scholar 

  • Bernsohn, J., and Grossman, H. J., eds., 1971, Lipid Storage Diseases: Enzymatic Defects and Clinical Implications, Academic Press, New York.

    Google Scholar 

  • Berthold, C.-H., 1973, Local “demyelination” in developing feline nerve fibres, Neurobiol. 3: 339–352.

    Google Scholar 

  • Beutler, E., and Kuhl, W., 1970, The diagnosis of the adult type of Gaucher’s disease and its carrier state by demonstration of deficiency of β-glucosidase activity in peripheral blood leukocytes, J. Lab. Clin. Med. 76: 747–755.

    PubMed  CAS  Google Scholar 

  • Bornstein, M. B., and Appel, S. H., 1961, The application of tissue culture to the study of experimental “allergic” encephalomyelitis. I. Patterns of demyelination, J. Neuropathol. Exptl. Neurol. 20: 141–157.

    CrossRef  Google Scholar 

  • Bowen, D. M., and Radin, N. S., 1968a, Purification of cerebroside galactosidase from rat brain, Biochim. Biophys. Acta 152: 587–598.

    PubMed  CAS  CrossRef  Google Scholar 

  • Bowen, D. M., and Radin, N. S., 1968b, Properties of cerebroside galactosidase, Biochim. Biophys. Acta 152: 599–610.

    PubMed  CAS  CrossRef  Google Scholar 

  • Bowen, D. M., and Radin, N. S., 1969a, Hydrolase activities in brain of neurological mutants</b>: Cerebroside galactosidase, nitrophenyl glucoside hydrolase, and sulfatase, J. Neurochem. 16: 457–460.

    Google Scholar 

  • Bowen D. M., and Radin, N. S., 1969b, Cerebroside galactosidase: A method for determination and a comparison with other lysosomal enzymes in developing rat brain, J. Neurochem. 16: 501–511.

    PubMed  CAS  CrossRef  Google Scholar 

  • Brady, R. O., Kanfer, J. N., Bradley, R. M., and Shapiro, D., 1966, Demonstration of a deficiency of glucocerebroside-cleaving enzyme in Gaucher’s disease, J. Clin. Invest. 45: 1112–1115.

    PubMed  CAS  CrossRef  Google Scholar 

  • Brady, R. O., Gal, A. E., Kanfer, J. N., and Bradley, R. M., 1965a, Purification and properties of a glucosyl- and galactosylceramide-cleaving enzyme from rat intestinal tissue, J. Biol. Chem. 240: 3766–3770.

    PubMed  CAS  Google Scholar 

  • Brady, R. O., Kanfer, J., and Shapiro, D., 1965b, Purification and properties of a glucocerebroside-cleaving enzyme from spleen tissue, J. Biol. Chem. 240: 39–43.

    PubMed  CAS  Google Scholar 

  • Brenkert, A., Arora, R. C, Radin, N. S., Meier, H., and MacPike, A. D., 1972, Cerebroside synthesis and hydrolysis in a neurological mutant mouse (msd), Brain Res. 36: 195–202.

    PubMed  CAS  CrossRef  Google Scholar 

  • Brosnan, C.F., Bunge, M. B., and Murray, M. R., 1970, The response of lysosomes in cultured neurons to chlorpromazine, J. Neuropath. Exp. Neurol. 29: 337–353.

    PubMed  CAS  CrossRef  Google Scholar 

  • Burton, R. M., and Guerra, F. C, eds., 1974, Fundamentals of Lipid Chemistry, BI-Science Publ. Div., Webster Groves, Mo.

    Google Scholar 

  • Curtino, J. A., and Caputto, R., 1974, Enzymic synthesis of cerebroside from glycosylsphingos-ine and stearoyl-CoA by an embryonic chicken brain preparation, Biochem. Biophys. Res. Commun. 56: 142–147.

    PubMed  CAS  CrossRef  Google Scholar 

  • Dawson, G., 1974, Glycolipid biosynthesis and catabolism, in Mammalian Glycoproteins and Glycolipids (W. Pigman and M. I. Horowitz, eds.), Academic Press, New York.

    Google Scholar 

  • Dawson, G., 1972, Glycosphingolipid levels in an unusual neurovisceral storage disease characterized by lactosylceramide galactosyl hydrolase deficiency: Lactosylceramidosis, J. Lipid Res. 13: 207–219.

    PubMed  CAS  Google Scholar 

  • Dawson, G., and Stein, A. O., 1970, Lactosyl ceramidosis: Catabolic enzyme defect of glyco- sphingolipid metabolism, Science 170: 556–558.

    PubMed  CAS  CrossRef  Google Scholar 

  • Dawson, G., and Sweeley, C. C., 1970, In vivo studies on glycosphingolipid metabolism in porcine blood, J. Biol. Chem. 245: 410–416.

    PubMed  CAS  Google Scholar 

  • Dawson, G., Stoolmiller, A. C, and Radin, N. S., 1974, Inhibition of β-glucosidase by N- (n-hexyl)-O-glucosylsphingosine in cell strains of neurological origin, J. Biol. Chem. 249: 4634–4646.

    Google Scholar 

  • DeVries, G. H., Norton, W. T., and Raine, C. S., 1972, Axons: Isolation from mammalian central nervous system, Science 175: 1370–1372.

    PubMed  CAS  CrossRef  Google Scholar 

  • Distler, J. J., and Jourdian, G. W., 1973, The purification and properties of β-galactosidase from bovine testes, J. Biol. Chem. 248: 6772–6780.

    PubMed  CAS  Google Scholar 

  • Erickson, J. S., and Radin, N. S., 1973, TV-Hexyl-O-glucosyl sphingosine, an inhibitor of glucosyl ceramide β-glucosidase, J. Lipid Res. 14: 133–137.

    PubMed  CAS  Google Scholar 

  • Evans, J. E., and McCluer, R. H., 1969, The structure of brain dihexosylceramide in globoid cell leukodystrophy, J. Neurochem. 16: 1393–1399.

    PubMed  CAS  CrossRef  Google Scholar 

  • Fredrickson, D. S., and Sloan, H. R., 1972, Glucosyl ceramide lipidoses: Gaucher’s disease, in The Metabolic Basis of Inherited Disease (J. B. Stanbury, J. B. Wyngaarden, and D. S. Fredrickson, eds.), pp. 730–759, McGraw-Hill Book Co., New York.

    Google Scholar 

  • Fry, J. M., Weissbarth, S., Lehrer, G. H., and Bornstein, M. B., 1974, Cerebroside antibody inhibits sulfatide synthesis and myelination and demyelinates in cord tissue cultures, Science 183: 540–542.

    PubMed  CAS  CrossRef  Google Scholar 

  • Gatt, S., 1966a, Hydrolysis and synthesis of ceramides by an enzyme from rat brain, J. Biol. Chem. 241: 3724–3730.

    PubMed  CAS  Google Scholar 

  • Gatt, S., 1966b, Enzymic hydrolysis of sphingolipids: Hydrolysis of ceramide glucoside by an enzyme from ox brain, Biochem. J. 101: 687–691.

    PubMed  CAS  Google Scholar 

  • Gatt, S., 1969a, β-Glucosidase from bovine brain, in Methods in Enzymology (J. M. Lowenstein, ed.), Vol. 14, pp. 152–155, Academic Press, New York.

    Google Scholar 

  • Gatt, S., 1969b, β-Galactosidase from rat brain, in Methods in Enzymology (J. M. Lowenstein, Vol. 14, pp. 157–158, Academic Press, New York.

    Google Scholar 

  • Gatt, S., 1970, Enzymatic estimation of galactose in the presence of galactolipids, Biochim. Biophys.Acta 218: 173–175.

    PubMed  CAS  CrossRef  Google Scholar 

  • Gatt, S., 1973, in Glycolipids, Glycoproteins, and Mucopolysaccharides of the Nervous System (V. Zambotti, G. Tettamanti, and M. Arrigoni, eds.), Plenum Press, New York.

    Google Scholar 

  • Gatt, S., and Barenholz, Y., 1973, Enzymes of complex lipid metabolism, Ann. Rev. Biochem. 42: 61–90.

    PubMed  CAS  CrossRef  Google Scholar 

  • Gatt, S., and Rapport, M. M., 1966, Hydrolysis of ceramide lactoside by an enzyme from rat brain, Biochem. J. 101: 680–686.

    PubMed  CAS  Google Scholar 

  • Gatt, S., and Yavin, E., 1969, Ceramidase from rat brain, in Methods in Enzymology (J. M. Lowenstein, ed.), Vol. 14, pp. 139–144, Academic Press, New York.

    Google Scholar 

  • Glew, R. H., and Lee, R. E., 1973, Composition of the membranous deposits occurring in Gaucher’s disease, Arch. Biochem. Biophys. 156: 626–639.

    PubMed  CAS  CrossRef  Google Scholar 

  • Glew, R. H., Christopher, A. R., Schnure, F. W., and Lee, R. E., 1974, The occurrence of beta-glucocerebrosidase activity in the glucocerebroside-rich deposits of Gaucher’s disease, Arch. Biochem. Biophys. 160: 162–167.

    PubMed  CAS  CrossRef  Google Scholar 

  • Gonzalez-Sastre, F., Pampols, T., and Sabater, J., 1974, Infantile Gaucher’s disease: A biochemical study, Neurology 24: 162–167.

    Google Scholar 

  • Hajra, A. K., and Radin, N. S., 1963, Isotopic studies of the biosynthesis of the cerebroside fatty acids in rats, J. Lipid Res. 4: 270–278.

    PubMed  CAS  Google Scholar 

  • Hajra, A. K., Bowen, D. M., Kishimoto, Y., and Radin, N. S., 1966, Cerebroside galactosidase of brain, J. Lipid Res. 7: 379–386.

    PubMed  CAS  Google Scholar 

  • Hammarström, S., 1971, A convenient procedure for the synthesis of ceramides, J. Lipid Res. 12: 760–765.

    PubMed  Google Scholar 

  • Hickman, S., Shapiro, L. J., and Neufeld, E. F., 1974, A recognition marker required for uptake of a lysosomal enzyme by cultured fibroblasts, Biochem. Biophys. Res. Commun. 57: 55–61.

    PubMed  CAS  CrossRef  Google Scholar 

  • Ho, M. W., 1973, Identity of “acid” β-glucosidase and glucocerebrosidase in human spleen, Biochem. 7.136: 721–729.

    Google Scholar 

  • Ho, M. W., and Light, N. D., 1973, Glucocerebrosidase: Reconstitution from macromolecular components depends on acidic phospholipids, Biochem. J. 136: 821–823.

    PubMed  CAS  Google Scholar 

  • Ho, M. W., and O’Brien, J. S., 1971, Gaucher’s disease: Deficiency of “acid” β-glucosidase and reconstitution of enzyme activity in vitro, Proc. Nat. Acad. Sci. (U.S.) 68: 2810–2813.

    Google Scholar 

  • Ho, M. W., O’Brien, J. S., Radin, N. S., and Erickson, J. S., 1973, Glucocerebrosidase</b>: Reconstitution of activity from macromolecular components, Biochem. J. 131: 173–176.

    PubMed  CAS  Google Scholar 

  • Hultberg, B., and φckerman, P. A., 1970, β-Glucosidase activities in human tissues; findings in Gaucher’s disease, Clin. Chim. Acta 28: 169–174.

    Google Scholar 

  • Hyun, J. C, and Radin, N. S., 1975, The assay of glucocerebroside β-glucosidase. Submitted for publication.

    Google Scholar 

  • Hyun, J. C, Misra, R. S., Greenblatt, D., and Radin, N. S., 1975, Synthetic inhibitors of glucocerebroside β-glucosidase. Arch. Biochem. Biophys. 166 (in press).

    Google Scholar 

  • Kampine, J. P., Kanfer, J. N., Gal, A. E., Bradley, R. M., and Brady, R. O., 1967, Response of sphingolipid hydrolases in spleen and liver to increased erythrocytorhexis, Biochim. Biophys. Acta 137: 135–139.

    PubMed  CAS  CrossRef  Google Scholar 

  • Kanfer, J. N., Stein, M., and Spielvogel, C, 1972, Recent observations on Gaucher’s disease, in Sphingolipids, Sphingolipidoses and Allied Disorders (B. W. Volk and S. M. Aronson, eds.), pp. 225–236, Plenum Press, New York.

    CrossRef  Google Scholar 

  • Kanfer, J. N., Petrovich, G., and Mumford, R. A., 1973, Purification of a- and β-galactosidases by affinity chromatography, Anal. Biochem. 55: 301–305.

    PubMed  CAS  CrossRef  Google Scholar 

  • Kanfer, J. N., Mumford, R. A., Raghavan, S. S., Friedman, R., and Byrd, J., 1974, Purification of β-glucosidase activities from bovine spleen by affinity chromatography, Federation Proc. 33: 1299.

    Google Scholar 

  • Kattlove, H. E., Williams, J. C, Gaynor, E., Spivack, M., Bradley, R. M., and Brady, R. O., 1969, Gaucher cells in chronic myelocytic leukemia: An acquired abnormality, Blood 33: 379–390.

    PubMed  CAS  Google Scholar 

  • Klibansky, Ch., Hoffman, J., Zaizov, R., and Matoth, Y., 1974, Gaucher’s disease, chronic adult type: A comparative study of glucocerebroside and methylumbelliferyl-glucopyran- oside cleaving potency in leukocytes, Biomed. 20: 24–30.

    CAS  Google Scholar 

  • Klibansky, Ch., Hoffman, J., Zaizov, R., Matoth, Y., Pinkhas, J., and de Vries, A., 1973, Chronic Gaucher’s disease: Heat-resistance of leukocyte glucocerebrosidase in relation to some clinical parameters, Biomed. 19: 345–348.

    CAS  Google Scholar 

  • Kopaczyk, K. C, and Radin, N. S., 1965, In vivo conversions of cerebroside and ceramide in rat brain, J. Lipid Res. 6: 140–145.

    PubMed  CAS  Google Scholar 

  • Kuske, T. T., and Rosenberg, A., 1972, Quantity and fatty acyl composition of the glycosphing- olipids of Gaucher spleen, J. Lab. Clin. Med. 80: 523–529.

    PubMed  CAS  Google Scholar 

  • Lai, H.-Y. L., Axelrod, B., 1973, 1-Aminoglycosides, a new class of specific inhibitors of glycos- idases, Biochem. Biophys. Res. Comm. 54: 463–468.

    PubMed  CAS  CrossRef  Google Scholar 

  • Lee, Y. C, 1972, Analysis of sugars by automated liquid chromatography, in Methods in Enzymology (V. Ginsburg, ed.), Vol. 28, pp. 63–73, Academic Press, New York.

    Google Scholar 

  • Leese, H. J., and Semenza, G., 1973, On the identity between the small intestinal enzymes phlorizin hydrolase and glycosylceramidase, J. Biol. Chem. 248: 8170–8173.

    PubMed  CAS  Google Scholar 

  • Li, S.-C, and Li, Y.-T., 1974, Isolation and characterization of a heat-stable glycoprotein activating the hydrolysis of sphingoglycolipids, Federation Proc. 33: 1299.

    Google Scholar 

  • Lin, Y.-N., and Radin, N. S., 1973, Alternate pathways of cerebroside catabolism, Lipids 8: 732–736.

    PubMed  CAS  CrossRef  Google Scholar 

  • Makita, A., Suzuki, C, Yosizawa, Z., and Konno, T., 1966, Glycolipids isolated from the spleen of Gaucher’s disease, J. Exptl. Med. 88: 277–288.

    CAS  CrossRef  Google Scholar 

  • Meisler, M., 1972, β-Galactosidase from human liver, in Methods in Enzymology (V. Ginsburg, ed.), Vol. 28, pp. 820–824, Academic Press, New York.

    Google Scholar 

  • Mellor, J. D., and Layne, D. S., 1971, Steroid β-D-glucosidase activity in rabbit tissues, J. Biol. Chem. 246: 4377–4380.

    PubMed  CAS  Google Scholar 

  • Mickel, H. S., and Gilles, F. H., 1970, Changes in glial cells during human telencephalic myelo- genesis, Brain 93: 337–346.

    PubMed  CAS  CrossRef  Google Scholar 

  • Miyatake, T., and Ariga, T., 1972, Sphingoglycolipids in the nervous system in Fabry’s disease, J, Neurochem. 19: 1911–1916.

    CAS  CrossRef  Google Scholar 

  • Miyatake, T., and Suzuki, K., 1974a, Glycosphingolipid β-galactosidases in rat brain</b>: Properties and the standard assay procedures of the enzymes in whole homogenate, Biochim. Biophys. Acta 337: 333–342.

    Google Scholar 

  • Miyatake, T., and Suzuki, K., 1974b, Galactosylsphingosine galactosyl hydrolase in rat brain: Probable identity with galactosylceramide galactosyl hydrolase, J. Neurochem. 22: 231–237.

    PubMed  CAS  CrossRef  Google Scholar 

  • Morell, P., and Braun, P., 1972, Biosynthesis and metabolic degradation of sphingolipids not containing sialic acid, J. Lipid Res. 13: 293–310.

    PubMed  CAS  Google Scholar 

  • Morell, P., and Radin, N. S., 1969, Synthesis of cerebroside by brain from uridine diphosphate galactose and ceramide-containing hydroxy fatty acid, Biochem. 8: 506–512.

    CAS  CrossRef  Google Scholar 

  • Morell, P., and Radin, N. S., 1970, Specificity in ceramide biosynthesis from long chain bases and various fatty acyl Coenzyme A’s by brain microsomes, J. Biol. Chem. 245: 342–350.

    PubMed  CAS  Google Scholar 

  • Morell, P., Costantino-Ceccarini, E., and Radin, N. S., 1970, The biosynthesis by brain microsomes of cerebrosides containing nonhydroxy fatty acids, Arch. Biochem. Biophys. 141: 738–748.

    PubMed  CAS  CrossRef  Google Scholar 

  • Moser, H. W., Prensky, A. L., Wolfe, H. J., and Rosman, N. P., 1969, Farber’s lipogranulomatosis : Report of a case and demonstration of an excess of free ceramide and ganglioside, Amer. J. Med. 47: 869–890.

    PubMed  CAS  CrossRef  Google Scholar 

  • Mraz, W., and Jatzkewitz, H., 1974, Cerebroside sulphatase activity of arylsulphatases from various invertebrates, Z. Physiol. Chem. 355: 33–44.

    CAS  CrossRef  Google Scholar 

  • Nilsson, Å., 1969, The presence of sphingomyelin- and ceramide-cleaving enzymes in the small intestinal tract, Biochim. Biophys. Acta 176: 339–347.

    PubMed  CAS  CrossRef  Google Scholar 

  • Ong, D. E., and Brady, R. N., 1972, Synthesis of ceramides using TV-hydroxysuccinimide esters, J. Lipid Res. 13: 819–822.

    PubMed  CAS  Google Scholar 

  • Patrick, A. D., 1965, A deficiency of glucocerebrosidase in Gaucher’s disease, Biochem. J. 97: 17c-18c.

    CAS  Google Scholar 

  • Pentchev, P. G., and Brady, R. O., 1973, The effect of a heat-stable factor in human spleen on glucocerebrosidase and acid β-glucosidase activities, Biochim. Biophys. Acta 297: 491–496.

    PubMed  CAS  CrossRef  Google Scholar 

  • Pentchev, P. G., Brady, R. O., Hibbert, S. R., Gal, A. E., and Shapiro, D., 1973, Isolation and characterization of glucocerebrosidase from human placental tissue, J. Biol. Chem. 248: 5256–5261.

    PubMed  CAS  Google Scholar 

  • Pentchev, P. G., Brady, R. O., Gal, A. E., Hibbert, S. R., and Dekaban, A. S., 1974, Infusion of purified glucocerebrosidase into patients with Gaucher’s disease, Federation Proc. 33: 1300.

    Google Scholar 

  • Pope, J. L., 1967, Crystallization of sodium taurocholate, J. Lipid Res. 8: 146–147.

    PubMed  CAS  Google Scholar 

  • Radin, N. S., 1970, Cerebrosides and sulfatides, in Handbook of Neurochemistry (A. Lajtha, ed.), Vol. 3, pp. 415–424, Plenum Press, New York.

    Google Scholar 

  • Radin, N. S., 1972a, Labeled galactosyl ceramide and lactosyl ceramide, in Methods in Enzymology (V. Ginsburg, ed.), Vol. 28, pp. 300–306, Academic Press, New York.

    Google Scholar 

  • Radin, N. S., 1972b, Galactosyl ceramide (galactocerebroside) β-galactosidase from brain, in Methods in Enzymology (V. Ginsburg, ed.), Vol. 28, pp. 834–839, Academic Press, New York.

    Google Scholar 

  • Radin, N. S., 1974, Preparation of psychosines (1–0-hexosylsphingosine) from cerebrosides, Lipids 9: 358–360.

    PubMed  CAS  CrossRef  Google Scholar 

  • Radin, N. S., and Arora, R. C, 1971, A simplified assay method for galactosyl ceramide β- galactosidase, J. Lipid Res. 12: 256–257.

    PubMed  CAS  Google Scholar 

  • Radin, N. S., Hof, L., Bradley, R. M., and Brady, R. O., 1969, Lactosylceramide galactosidase</b>: Comparison with other sphingolipid hydrolases in developing rat brain, Brain Res. 14: 497–505.

    Google Scholar 

  • Radin, N. S., Brenkert, A., Arora, R. C, Sellinger, O. Z., and Flangas, A. L., 1972a, Glial and neuronal localization of cerebroside-metabolizing enzymes, Brain Res. 39: 163–169.

    PubMed  CAS  CrossRef  Google Scholar 

  • Radin, N. S., Arora, R. C, Ullman, M. D., Brenkert, A. L., and Austin, J., 1972b, A possible therapeutic approach to Krabbe’s globoid leukodystrophy and the status of cerebroside synthesis in the disorder, Res. Comm. Chem. Pathol. Pharmacol. 3: 637–644.

    CAS  Google Scholar 

  • Raghavan, S. S., Mumford, R. A., and Kanfer, J. N., 1973, Deficiency of glucosylsphingosine β-glucosidase in Gaucher’s disease, Biochem. Biophys. Res. Commun. 54: 256–263.

    PubMed  CAS  CrossRef  Google Scholar 

  • Raghavan, S. S., Mumford, R. A., and Kanfer, J. N., 1974, Synthesis of glucosylceramide by purified calf spleen β-glucosidase, Biochem. Biophys. Res. Commun. 58: 99–106.

    PubMed  CAS  CrossRef  Google Scholar 

  • Read, W. K., and Bay, W. W., 1971, Basic cellular lesion in chloroquine toxicity, Lab. Invest. 24: 246–259.

    PubMed  CAS  Google Scholar 

  • Reese, E. T., Parrish, F. W., and Ettlinger, M., 1971, Nojirimycin and D-glucono-l,5-lactone as inhibitors of carbohydrases, Carbohyd. Res. 18: 381–388.

    CAS  CrossRef  Google Scholar 

  • Rosenberg, A., 1970, Sphingomyelin: enzymatic reactions, in Handbook of Neurochemistry (A. Lajtha, ed.), Vol. 3, pp. 453–466, Plenum Press, New York.

    Google Scholar 

  • Shapiro, D., 1969, Chemistry of Sphingolipids, Hermann Publ., Paris.

    Google Scholar 

  • Sourander, P., Hansson, H.-A., Olsson, Y., and Svennerholm, L., 1966, Experimental studies on the pathogenesis of leukodystrophies. II. The effect of sphingolipids on various cell types in cultures from the nervous system, Acta Neuropath. 6: 231–242.

    PubMed  CAS  CrossRef  Google Scholar 

  • Subba Rao, K., Wenger, D. A., and Pierenger, R. A., 1970, The metabolism of glyceride glyco- lipids. IV. Enzymatic hydrolysis of monogalactosyl and digalactosyl diglycerides in rat brain, J. Biol. Chem. 245: 2520–2524.

    PubMed  CAS  Google Scholar 

  • Sugita, M., Dulaney, J. T., and Moser, H. W., 1972, Ceramidase deficiency in Farber’s disease (lipogranulomatosis), Science 178: 1100–1102.

    PubMed  CAS  CrossRef  Google Scholar 

  • Sugita, M., Connolly, P., Dulaney, J. T., and Moser, H. W., 1973, Fatty acid composition of free ceramides of kidney and cerebellum from a patient with Farber’s disease, Lipids 8: 401–406.

    PubMed  CAS  CrossRef  Google Scholar 

  • Suzuki, K., and Suzuki, Y., 1972, Galactosyl ceramide lipidosis: globoid cell leukodystrophy (Krabbe’s disease), in The Metabolic Basis of Inherited Disease (J. B. Stanbury, J. B. Wyngaarden, and D. S. Fredrickson, eds.), pp. 760–782, McGraw-Hill Book Co., New York.

    Google Scholar 

  • Suzuki, K., Suzuki, Y., and Fletcher, T. F., 1972, in Sphingolipids, Sphingolipidoses and Allied Disorders (B. W. Volk and S. M. Aronson, eds.), pp. 478–498, Plenum Press, New York.

    Google Scholar 

  • Suzuki, Y., and Suzuki, K., 1974a, Glycosphingolipid β-galactosidases. I. Standard assay procedures and characterization by electrofocusing and gel filtration of the enzymes in normal human liver, J. Biol. Chem. 249: 2098–2104.

    PubMed  CAS  Google Scholar 

  • Suzuki, Y., and Suzuki, K., 1974b, Glycosphingolipid β-galactosidases. II. Electrofocusing characterization of the enzymes in human globoid cell leukodystrophy (Krabbe’s disease), J. Biol. Chem. 249: 2105–2108.

    PubMed  CAS  Google Scholar 

  • Suzuki, Y., Miyatake, T., Fletcher, T. F., and Suzuki, K., 1974, Glycosphingolipid β-galactosid- ases. III. Canine form of globoid cell leukodystrophy ; comparison with the human disease, J. Biol. Chem. 249: 2109–2112.

    PubMed  CAS  Google Scholar 

  • Svennerholm, L., 1966, The metabolism of gangliosides in cerebral lipidoses, in Inborn Disorders of Sphingolipid Metabolism (S. M. Aronson and B. W. Volk, eds.), p. 173, Pergamon Press, New York.

    Google Scholar 

  • Taketomi, T., and Yamakawa, T., 1967, Glycolipids of the brain in gargoylism, Jap. J. Exptl. Med. 37: 11–21.

    CAS  Google Scholar 

  • Ullman, M. D., and Radin, N. S., 1972, Enzymatic formation of hydroxy ceramides and comparison with enzymes forming nonhydroxy ceramides, Arch. Biochem. Biophys. 152: 767 – 777.

    PubMed  CAS  CrossRef  Google Scholar 

  • Volk, B. W., and Aronson, S. M., eds., 1972, Sphingolipids, Sphingolipidoses and Allied Disorders, Plenum Press, New York.

    Google Scholar 

  • Warren, K. R., Schafer, I. A., Sullivan, J. C, Petrelli, M., and Radin, N. S., 1974, Induction of a chemical model of Gaucher’s disease in normal cultured human fibroblasts. Submitted for publication.

    Google Scholar 

  • Weinreb, N. J., and Brady, R. O., 1972, Glucocerebrosidase from bovine spleen, in Methods in Enzymology (V. Ginsburg, ed.), Vol. 28, pp. 830–834, Academic Press, New York.

    Google Scholar 

  • Weiss, B., and Raizman, P., 1958, Synthesis of long chain fatty acid amides of sphingosine and dihydrosphingosine, J. Am. Chem. Soc. 80: 4657–4658.

    CAS  CrossRef  Google Scholar 

  • Wenger, D. A., Sattler, M., and Markey, S. P., 1973, Deficiency of monogalactosyl diglyceride β-galactosidase activity in Krabbe’s disease, Biochem. Biophys. Res. Commun. 53: 680–685.

    PubMed  CAS  CrossRef  Google Scholar 

  • Wenger, D. A., Sattler, M., and Hiatt, W., 1974, Globoid cell leukodystrophy: deficiency of lactosyl ceramide beta-galactosidase, Proc. Nat. Acad. Sci. (U.S.) 71: 854–857.

    CAS  CrossRef  Google Scholar 

  • Yavin, E., and Gatt, S., 1969, Enzymatic hydrolysis of sphingolipids. VIII. Further purification and properties of rat brain ceramidase, Biochemistry 8: 1692–1698.

    PubMed  CAS  CrossRef  Google Scholar 

  • Yunis, E. J., and Lee, R. E., 1970, Tubules of globoid leukodystrophy: A right-handed helix, Science 169: 64–66.

    PubMed  CAS  CrossRef  Google Scholar 

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Radin, N.S. (1975). Hydrolases of the Simple Sphingolipids: Ceramide, Glucocerebroside, and Galactocerebroside. In: Agranoff, B.W., Aprison, M.H. (eds) Advances in Neurochemistry. Springer, Boston, MA. https://doi.org/10.1007/978-1-4757-4395-1_2

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