Summary
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1.
The activity of the sulphotransferase involved in the synthesis of sulphatides, 3′-phosphoadenosine-5′-phosphosulphate cerebroside sulphotransferase (PAPS-CST), was determined in the developing CNS of the myelin synthesis deficiency mutant mouse (msd) and compared to that in control animals. There was a decrease of the PAPS-CST activity in msd mouse in the period from 8 to 26 days. The developmental curve of PAPS-CST activity in msd mice brains essentially paralleled that in normal animals, with a maximum at about 19 days.
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2.
Basic enzyme properties, such as Km, optimum pH, and heat inactivation, were not affected by the mutation.
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Abbreviations
- PAPS-CST:
-
3′-phosphoadenosine-5′-phosphosulphate-cerebroside sulphotransferase
- ATP:
-
adenosine triphosphate
- NFA:
-
non hydroxy fatty acids
- HFA:
-
hydroxy fatty acids
References
Balasubramanian, A.S., Bachhawat, B.K.: Formation of cerebroside sulphate from 3′phosphoadenosine-5′-phosphosulphate in sheep brain. Biochim. biophys. Acta (Amst.) 106, 218–220 (1965)
Basu, S., Kaufman, B., Roseman, S.: Enzymatic synthesis of ceramide-glucose and ceramidelactose by glycosyl-transferases from embryonic chicken brain. J. biol. Chem. 243, 5802–5807 (1968)
Bhandari, V.R., Bachhawat, B.K.: Biosynthesis of cerebroside sulphate in brain: Part I. Studies on the nature of the endogenous sulphate acceptor from sheep brain. Indian. J. Biochem. 9, 72–77 (1972)
Berger, B.: Quelques aspects ultrastructuraux de la substance blanche chez la souris Quaking. Brain Res. 25, 35–53 (1971)
Bosch, M.A., Rebel, G., Sarliève, L.L., Neskovic, N.M.: Brain gangliosides of myelin synthesis deficient mice. Lipids 8, 436–438 (1973)
Brenkert, A., Arora, R.C., Radin, N.S., Meier, H., MacPike, A.D.: Cerebroside synthesis and hydrolysis in a neurological mutant mouse (msd). Brain Res. 36, 195–202 (1972)
Costantino-Ceccarini, E., Morell, P.: Quaking mouse: in vitro studies of brain sphingolipid biosynthesis. Brain Res. 29, 75–84 (1971)
Desmukh, D.S., Inoue, T., Pieringer, R.A.: The association of the galactosyl diglycerides of brain with myelination. II. The inability of the myelin-deficient mutant, Jimpy mouse to synthesize galactosyl diglycerides effectively. J. biol. Chem. 246, 5695–5699 (1971)
Druse, M.J., Hogan, E.L.: Characterization of myelin proteins in the Jimpy mutant. Trans. Amer. Soc. Neurochem. 3, 73 (1972)
Farkas-Bargeton, E., Robain, O., Mandel, P.: Abnormal glial maturation in the white matter in Jimpy mice. An optical study. Acta neuropath. (Berl.) 21, 272–281 (1972)
Friedrich, V.L., Jr., Hauser, G.: Biosynthesis of psychosine and levels of cerebrosides in the central and peripheral nervous systems of Quaking mice. J. Neurochem. 20, 1131–1141 (1973)
Greenfield, S., Norton, W.T., Morell, P.: Quaking mouse: isolation and characterization of myelin protein. J. Neurochem. 18, 2119–2128 (1971)
Herschkowitz, N., Vassella, F.: Myelin differences in the central and peripheral nervous system in the Jimpy mouse. J. Neurochem. 18, 1361–1363 (1971)
Kandutsch, A.A., Saucier, S.E.: Regulation of sterol synthesis in developing brains of normal and Jimpy mice. Arch. Biochem. Biophys. 135, 201–208 (1969)
—: Sterol and fatty acid synthesis in developing brains of three myelin-deficient mouse mutants. Biochim. biophys. Acta (Amst.) 260, 26–34 (1972)
Kanfer, J., Stein, M.: Sulfatide biosynthesis by intact microsomes and Triton extracts of normal and “Quaking” mouse brain. Lipids 7, 259–261 (1972)
Kostic, D., Nussbaum, J.L., Mandel, P.: A study of brain gangliosides in Jimpy mutant mice. Life Sci. 8, 1135–1143 (1969)
— Neskovic, N.: Les gangliosides du cerveau de deux mutants “Quaking” et “Jimpy”. Acta med. jugosl. 24, 20–26 (1970)
Mandel, P., Nussbaum, J.L., Neskovic, N.M., Sarliève, L.L., Kurihara, T.: Regulation of myelinogenesis. In: Adv. Enzyme Regul, pp. 101–118. Ed. by G. Weber, New York: Pergamon Press 1972
Matthieu, J.M., Schneider, U., Herschkowitz, N.: In vitro synthesis of sulfatide in a myelin deficient mutant: the Jimpy mouse. Brain Res. 42, 433–439 (1972)
McKhann, G.M., Levy, R., Ho, W.: Metabolism of sulfatides. I. The effect of galactocerebrosides on the synthesis of sulfatides. Biochem. biophys. Res. Commun. 20, 109–113 (1965)
Meier, H., Dickie, M.M.: A neurological mutation of the mouse with a deficiency of myelin synthesis. Abstr. 2nd Meeting Intern. Soc. Neurochem. Milan. p. 287, 1969
— MacPike, A.D.: A neurological mutation (msd) of the mouse causing a deficiency of myelin synthesis. Exp. Brain Res. 10, 512–525 (1970)
Neskovic, N.M., Nussbaum, J.L., Mandel, P.: Enzymatic synthesis of psychosine in “Jimpy” mice brain. FEBS Lett. 3, 199–201 (1969a)
—: Etude de la galactosyl-sphingosine transférase du cerveau de souris mutante “Quaking”. C. R. Acad. Sci. (Paris) Sér. D 269, 1125–1128 (1969b)
—: Enzymatic deficiency in neurological mutants. Brain uridine diphosphate galactose: ceramide galactosyl transferase in Jimpy mouse. FEBS Lett. 8, 213–216 (1970a)
—: A study of glycolipid metabolism in myelination disorder of Jimpy and Quaking mice. Brain Res. 21, 39–53 (1970)
— Sarliève, L.L., Mandel, P.: Biosynthesis of glycolipids in myelin deficient mutants. Brain glycosyltransferases in Jimpy and Quaking mice. Brain Res. 42, 147–157 (1972)
—: Subcellular and submicrosomal distribution of glycolipid-synthesizing transferases in young rat brain. J. Neurochem. 20, 1419–1430 (1973)
Nussbaum, J.L., Mandel, P.: Brain proteolipids in neurological mutant mice. Brain Res. 61, 295–310 (1973)
Philippart, M., Sarliève, L., Meurant, C., Mechler, L.: Human urinary sulfatides in patients with sulfatidosis (metachromatic leukodystrophy). J. Lipid Res. 12, 434–441 (1971)
Radin, N.S., Brenkert, A., Arora, R.C., Sellinger, O.Z., Flangas, A.L.: Glial and neuronal localization of cerebroside-metabolizing enzymes. Brain Res. 39, 163–169 (1972)
Sarliève, L.L., Neskovic, N.M., Mandel, P.: PAPS-cerebroside sulphotransferase activity in brain and kidney of neurological mutants. FEBS Lett. 19, 91–95 (1971)
— Rebel, G., Mandel, P.: Some properties of brain PAPS-cerebroside sulphotransferase in Jimpy and Quaking mice. Neurobiology 2, 70–82 (1972)
Saxena, S., George, E., Kokrady, S., Bachhawat, B.K.: Sulphate metabolism in developing rat brain. A study with subcellular fractions. Indian J. Biochem. 8, 1–8 (1971)
Schneider, P.B., Kennedy, E.P.: Metabolism of labelled dihydrosphingomyelin in vivo. J. Lipid Res. 9, 58–64 (1968)
Stoffyn, P., Stoffyn, A., Hauser, G.: Structure of sulfatides biosynthesized in vitro. J. Lipid Res. 12, 318–323 (1971)
Svennerholm, L.: The quantitative estimation of cerebrosides in nervous tissue. J. Neurochem. 1, 42–53 (1956)
—: Gangliosides. In: Handbook of Neurochemistry, vol. III, p. 425–452. Ed. by A. Lajtha, New York: Plenum Press 1970
Waehneldt, T.V., Neuhoff, V.: Membrane proteins of the nervous system. Demonstration of different protein profiles in whole brain and its subcellular particles. Naturwissenschaften 59, 232–239 (1972)
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With the technical assistance of P. Guerin and M. Perraut.
Attaché de Recherche à l'INSERM.
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Sarlieve, L.L., Neskovic, N.M., Rebel, G. et al. PAPS-cerebroside sulphotransferase activity in developing brain of a neurological mutant of mouse (msd). Exp. Brain Res. 19, 158–165 (1974). https://doi.org/10.1007/BF00238532
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DOI: https://doi.org/10.1007/BF00238532