Abstract
The lysosomal phospholipid, bis(monoacylglycero)phosphate1 (BMP), accumulates selectively in both inherited2–4 and acquired5-6 disorders of lysosomal lipid catabolism. In those forms of Niemann-Pick disease with a profound deficiency of lysosomal sphingomyelinase, there is also an equally profound deficiency of phospholipase C7. Although sphingomyelin and phospholipids are substrates for the defective enzyme, there may be greater relative increase of BMP than of sphingomyelin and both sphingomyelin and BMP accumulate to a much greater degree than the other phospholipids. Lack of accumulation of phospholipids has been explained by the availability of other pathways excusive of phospholipase C for catabolism that are assumed to be unavailable to either sphingomyelin or BMP7.
Access this chapter
Tax calculation will be finalised at checkout
Purchases are for personal use only
Preview
Unable to display preview. Download preview PDF.
References
J. R. Wherrett and S. Huterer, Enrichment of bis(monoacylglyceryl)-phosphate in lysosomes from rat liver, J. Biol. Chem. 247:411 (1972).
G. Rouser, G. Kritchevsky, A. Yamamoto and G. Simon, Accumulation of glycerolphospholipid in classical Niemann-Pick disease, Lipids 3:287 (1968).
J. R. Wherrett and N. B. Rewcastle, Adult neurovisceral lipidosis, Clin. Res. 17:665 (1969).
P. N. Seng, H. Debuch, B. Witter and H. R. Wiedemann, Bis(monoacylglycerin) phosphasaure-Vermehrung bei Sphingomyelinase (M. Niemann-Pick?) Hoppe-Seyler’s Z. Physiol. Chem. 352:280 (1971).
S. Adachi, Y. Matsuzawa, T. Yokomura, K. Ishikawa S. Uhara, A. Yamamoto and M. Nishikawa, Changes in the lipid composition of rat liver and spleen following the administration of 4,4-dimethylaminoethoxyhexestrol, Lipids 7:1 (1972).
H. Lullmann and R. Lullmann-Rauch and O. Wassermann, Drug-induced phospholipidosis, Crit. Rev. Toxicol. 4:185 (1975).
S. Huterer, J. R. Wherrett, A. Poulos and J. W. Callahan, Deficiency of phospholipase C acting on phosphatidylglycerol in Niemann-Pick disease, Neurology (NY) 33:67 (1983).
S. Huterer and J. R. Wherrett, Metabolism of bis(monoacylglycero)-phosphate in macrophages, J. Lipid Res. 20:966 (1979).
S. Huterer and J. R. Wherrett, Incorporation of polyunsaturatd fatty acids in bis(monoacylglycero)phosphate and other lipids of macrophage! and of fibroblasts from control and Niemann-Pick patients, Biochim. Biophys. Acta 876:318 (1985).
P-Y. Tsai and R. P. Geyer, Effect of exogenous fatty acids on the retention of phospholipid acyl groups by mouse L fibroblasts, Biochim Biophys. Acta 528:344 (1978).
A. A. Spector, S. N. Mathur, T. L. Kaduce and B. T. Hyman, Lipid nutrition and metabolism of cultured mammalian cells, Prog. Lipid Res 19:155 (1981).
R. J. Mason, T. P. Stossei and M. Vaughan, Lipids of alveolar macrophages, polymorphonuclear leukocytes, and their phagocytic vesicles, J. Clin. Invest. 51:2399 (1972).
J. Brotherus, O. Renkonen, J. Herrmann and W. Fischer, Novel stereoconfiguration in lyso-bis-phosphatidic acid of cultured BHK-cells, Chem. Phys. Lipids 13:178 (1974).
F. R. Cochran, V. L. Roddick, J. R. Connor, J. R. Thornburg and M. Waite, Regulation of arachidonic acid metabolism in resident and BCG-activated alveolar macrophages: role of lyso(bis)phosphatidic acid, J. Immunol. 138:1877 (1987).
J. Brotherus, T. Niinioja, K. Sandelin and O. Renkonen, Experimentall caused proliferation of lysosomes in cultured BHK cells involving an increase of biphosphatidic acids and triglycerides, J. Lipid Res. 18: 379 (1977).
S. Albouz, J-M. Boutry, G. Dubois, R. Bourdon, J-J. Hauw and N. Baumann, Lipid and lysosomal exzymes in human fibroblasts cultured with perhexiline maleate, Naunyn-Schmiedeberg’s Arch. Pharmacol. 317:173 (1981).
G. Albert-Tulkens, F. Van Hoof and P. Tulkens, Gentamicin-induced lysosomal phospholipidosis in cultured rat fibroblasts, Lab. Invest. 40:481 (1979).
H. S. Kruth, M. E. Comly, J. D. Butler, M. T. Vanier, J. K. Fink, D. A. Wenger, S. Patel and P. G. Pentchew, Type C Niemann-Pick disease: abnormal metabolism of low density lipoprotein in homozygous and heterozygous fibroblasts, J. Biol. Chem. 261:16769 (1986).
Author information
Authors and Affiliations
Editor information
Editors and Affiliations
Rights and permissions
Copyright information
© 1988 Plenum Press, New York
About this paper
Cite this paper
Huterer, S., Wherrett, J. (1988). Turnover of Docosahexaenoic Acid in Bis(Monoacylglycero)Phosphate Induced in Niemann-Pick Fibroblasts by Incubation in the Presence of Excess Fatty Acid. In: Salvayre, R., Douste-Blazy, L., Gatt, S. (eds) Lipid Storage Disorders. NATO ASI Series, vol 150. Springer, Boston, MA. https://doi.org/10.1007/978-1-4613-1029-7_16
Download citation
DOI: https://doi.org/10.1007/978-1-4613-1029-7_16
Publisher Name: Springer, Boston, MA
Print ISBN: 978-1-4612-8300-3
Online ISBN: 978-1-4613-1029-7
eBook Packages: Springer Book Archive