The immunoblotting technique was used to identify sphingomyeJinase protein in samples of tissue and urine after subjection to poIyacrylamide-gel etectrophoresis in the presence of sodium dodecyl sulphate. In a sphingomyelinase preparation purified from control urine a prominent band was seen with an Mr of 28 000 Da. Glycoprotein fractions from urine and placenta, a membrane extract from spleen, and a partially purified sphingomyelinase preparation from placenta contained the 28 000-Da band plus additional, higher-Mr bands. The 28 000-Da band was detectable in urine from a patient with Niemann-Pick disease type C, but not in urine from patients with Niemann-Pick disease types A and B. It is concluded that sphingomyeJinase is composed of at least one polypeptide with an Mr of 28 000 Da and that this polypeptide is deficient in the urine of patients with Niemann-Pick disease types A and B.
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December 01 1984
Immunological studies on lysosomal sphingomyelinase: Identification of a 28 000-Da component deficient in urine from patients with Niemann-Pick disease types A and B
André W. Schram;
André W. Schram
1Section for Medical Enzymology and Metabolism, Laboratory of Biochemistry, University of Amsterdam, P.O. Box 20151, 1000 HD Amsterdam, The Netherlands
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Marianne Dreissen;
Marianne Dreissen
1Section for Medical Enzymology and Metabolism, Laboratory of Biochemistry, University of Amsterdam, P.O. Box 20151, 1000 HD Amsterdam, The Netherlands
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Johan Bastiaannet;
Johan Bastiaannet
1Section for Medical Enzymology and Metabolism, Laboratory of Biochemistry, University of Amsterdam, P.O. Box 20151, 1000 HD Amsterdam, The Netherlands
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Wilma E. Donker-Koopman;
Wilma E. Donker-Koopman
1Section for Medical Enzymology and Metabolism, Laboratory of Biochemistry, University of Amsterdam, P.O. Box 20151, 1000 HD Amsterdam, The Netherlands
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Elisabeth M. Brouwer-Kelder;
Elisabeth M. Brouwer-Kelder
1Section for Medical Enzymology and Metabolism, Laboratory of Biochemistry, University of Amsterdam, P.O. Box 20151, 1000 HD Amsterdam, The Netherlands
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Gabriele Weitz;
Gabriele Weitz
2Institute of Organic Chemistry and Biochemistry, University of Bonn, Gerhard-Domagkstrasse 1, D-5300 Bonn, Germany
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John A. Barranger;
John A. Barranger
3Developmental and Metabolic Neurology Branch, National Institute of Neurological and Communicative Diseases and Stroke, National Institutes of Health, Bethesda, MD 20205, USA
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Konrad Sandhoff;
Konrad Sandhoff
2Institute of Organic Chemistry and Biochemistry, University of Bonn, Gerhard-Domagkstrasse 1, D-5300 Bonn, Germany
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Joseph M. Tager
Joseph M. Tager
1Section for Medical Enzymology and Metabolism, Laboratory of Biochemistry, University of Amsterdam, P.O. Box 20151, 1000 HD Amsterdam, The Netherlands
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Publisher: Portland Press Ltd
Received:
November 01 1984
Online ISSN: 1573-4935
Print ISSN: 0144-8463
© 1984 The Biochemical Society
1984
Biosci Rep (1984) 4 (12): 1051–1057.
Article history
Received:
November 01 1984
Citation
André W. Schram, Marianne Dreissen, Johan Bastiaannet, Wilma E. Donker-Koopman, Elisabeth M. Brouwer-Kelder, Gabriele Weitz, John A. Barranger, Konrad Sandhoff, Joseph M. Tager; Immunological studies on lysosomal sphingomyelinase: Identification of a 28 000-Da component deficient in urine from patients with Niemann-Pick disease types A and B. Biosci Rep 1 December 1984; 4 (12): 1051–1057. doi: https://doi.org/10.1007/BF01116698
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