Abstract
The relative amount of lysobisphosphatidic acid (LBPA), known also as bis(monoacylglyceryl)phosphate, among the total phospholipids was analyzed in post mortem samples of brain and liver of patients affected by four storage diseases. In spite of the extensive accumulation of storage lysosomes, none of the samples revealed a highly elevated LBPA content comparable to that found in the liver in Niemann-Pick disease and in the liver in lipidosis induced by 4,4′-diethylaminoethoxyhexestrol. We conclude that, although LBPA is often present in high concentration in lysosomes of many types of cells, it is not always a major component of these organelles.
Similar content being viewed by others
References
Brotherus, J., O. Renkonen, J. Herrmann, and W. Fischer, Chem. Phys. Lipids 13:178 (1974).
Body, D.R., and G.M. Gray, Ibid. 1:245 (1967).
Rouser, G., G. Simon, and G. Kritchevsky, Lipids 4:599 (1969).
Simon, G., and G. Rouser, Ibid. 4:607 (1969).
Brotherus, J., and O. Renkonen, Chem. Phys. Lipids 13:11 (1974).
Wherrett, J.R., and S. Huterer, J. Biol. Chem. 247:4114 (1972).
Mason, R.J., T.P. Stossel, and M. Vaughan, J. Clin. Invest. 51:2399 (1972).
Brotherus, J., I. Virtanen, and O. Renkonen, 12th International Conference for Fat Research, Milan, 1974, Abstr. 266.
Zeman, W., and A.N. Siakotos, in “Lysosomes and Storage Disease”, Edited by H.G. Hers and F. van Hoof, Academic Press, New York and London, 1973, p. 519.
Siakotos, A.N., G. Rouser, and S. Fleischer, Lipids 4:234 (1969).
Rouser, G., and S. Fleischer, Ibid. 4:239 (1969).
Fleischer, B., and S. Fleischer, in “Biomembranes”, Vol. II, Edited by L. Manson, Academic Press, New York and London, 1971, p. 75.
Renkonen, O., A. Luukkonen, J. Brotherus, and L. Kääriäinen, in “Control of Proliferation in Animal Cells”, Edited by B. Clarkson and R. Baserga, Cold Spring Harbor Laboratory, Cold Spring Harbor, NY 1974, p. 495.
Hers, H.G., in “Lysosomes and Storage Diseases”, Edited by H.G. Hers, and F. van Hoof, Academic Press, New York and London, 1973, p. 147.
Rouser, G., G. Kritchevsky, A. Yamamoto, A.G. Knudson, and G. Simon, Lipids 3:287 (1968).
Kamoshita, S., A.M. Aron, K. Suzuki, and K. Suzuki, Am. J. Dis. Child. 117:379 (1969).
Seng, P.N., H. Debuch, B. Witter, and H.R. Wiedemann, Z. Physiol. Chem. 352:280 (1971).
Martin, J.-J., M. Philippart, J.V. Callahan, and R. Deberdt, Arch. Neurol. 27:45 (1972).
Yamamoto, A., S. Adachi, K. Ishikawa, T. Yokomura, T. Kitani, T. Nasu, T. Imoto, and M. Nishikawa, J. Biochem. 70:775 (1971).
Adachi, S., Y. Matsusawa, T. Yokomura, K. Ishikawa, S. Uhara, A. Yamamoto, and M. Nichikawa, Lipids 7:1 (1972).
Kasama, K., K. Yoshida, S. Takeda, S. Akeda, and K. Kawai, Ibid., 9:235 (1974).
Haltia, M., J. Rapola, and P. Santavuori, Acta Neuropathol. 26:157 (1973).
Haltia, M., J. Rapola, P. Santavuori, and A. Keränen, J. Neurol. Sci. 18:269 (1973).
Svennerholm, L., B. Hagberg, M. Haltia, P. Sourander, and M.-T. Vanier, Acta Paediatr. Scand. 64:489 (1975).
Zeman, W., S. Donahue, P. Dyken, and J. Green, in “Hanbook of Clinical Neurology”, Vol. 10, Edited by P.J. Vinken and G.W. Gruyn, North Holland Publishing Co., Amsterdam, The Netherlands, 1970, p. 588.
Palo, J., “Occurrence of Metabolic Disorders in Developmental Malfunctions of the Central Nervous System” (in Finnish), PhD Thesis, University of Helsinki, Helsinki, Finland, 1966, p. 46.
Jenner, F.A., and R.J. Pollitt, Biochem. J. 48P:103 (1967).
Palo, J., R.J. Pollitt, K.M. Pretty, and H. Savolainen, Clin. Chim. Acta 47:69 (1973).
Haltia, M., J. Palo, and S. Autio, Acta Neuropathol. 31:243 (1975).
O’Brien, J.S., in “Lysosomes and Storage Diseases”, Edited by H.G. Hers and F. van Hoff, Academic Press, New York and London, 1973, p. 323.
Sandhoff, K., and K. Harzer, Ibid. “ p. 345.
Palo, J., M. Haltia, and G. Amnell, Duodecim 91:354 (1975).
Kates, M. “Techniques of Lipidology”, North Holland Publishing Co., Amsterdam, The Netherlands, 1972, p. 349.
Dittmer, J.C., and R.L. Lester, J. Lipid Res. 5:126 (1964).
Barlett, G.R., J. Biol. Chem. 234:466 (1959).
Dawson, R.M.C., Biochem. J. 75:45 (1960).
Rouser, G., G.J. Nelson, S. Fleischer, and G. Simon, in “Biologcal Membranes”, Edited by D. Chapman, Academic Press, New York and London, 1968, p. 1.
Author information
Authors and Affiliations
About this article
Cite this article
Kahma, K., Brotherus, J., Haltia, M. et al. Low and moderate concentrations of lysobisphosphatidic acid in brain and liver of patients affected by some storage diseases. Lipids 11, 539–544 (1976). https://doi.org/10.1007/BF02532899
Received:
Issue Date:
DOI: https://doi.org/10.1007/BF02532899