Summary
Two enzymes, lipoprotein lipase and hepatic triglyceride lipase, are involved in the hydrolysis of triglycerides from chylomicrons and very low density lipoprotein (VLDL). Lipoprotein lipase has an absolute requirement for apolipoprotein CII for activity. Three inborn errors of metabolism which give rise to hypertriglyceridaemia have been described. The biochemical and clinical aspects of these disorders, lipoprotein lipase deficiency (familial type I hyperlipoproteinaemia), hepatic triglyceride lipase deficiency and apo-CII deficiency are discussed.
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References
Augustin, J. and Greten, H. In Paoletti, R. and Golto, A.M. (eds.) Atherosclerosis Reviews, Vol. 5, Raven Press, New York (1979).
Breckenridge, W. C., Little, J. A., Steiner, G., Chow, A. and Poapst, M. Hypertriglyceridaemia associated with deficiency of apolipoprotein C-II. N. Engl. J. Med. 298 (1978) 1265–1273
Breckenridge, W. C., Little, J. A., Alaupovic, P., Wang, C. S., Kuksis, A., Kakis, G., Lindgrene, F. and Gardner, G. Lipoprotein abnormalities associated with a familial deficiency of hepatic lipase. Atherosclerosis 45 (1982) 161–179
Connelly, P. W., Maguire, G. F. and Little, J. A. Apolipoprotein C-II St Michael. Familial apolipoprotein C-II deficiency associated with premature vascular disease. J. Clin. Invest. 80 (1987) 1597–1606
Greten, H., de Gretta, R., Klose, G., Roscher, W., deGennes, J. L. and Gjone, E. Measurement of two plasma triglyceride lipases by an immunochemical method: studies in patients with hypertriglycerdaemia. J. Lipid Res. 17 (1976) 203–210
Klose, G., Windelband, J., Weizel, A. and Greten, H. Secondary hypertriglyceridaemia in patients with parenchymal liver disease. Eur. J. Clin. Invest. 1 (1977) 557–562
LaRosa, J. C., Levy, R. I., Herbert, P., Lux, S. E. and Frederickson, D. S. A specific apolipoprotein activator for lipoprotein lipase. Biochem. Biophys. Res. Commun. 41 (1970) 57–62
Nikkilä, F. A. Familial lipoprotein lipase deficiency and related disorders of chylomicron metabolism. In Stanbury, J. B., Wyngaarden, J. B., Frederickson, D. S., Goldstein, J. L. and Brown, M. S. (eds.) The Metabolic Basis of Inherited Disease. 5th Edn., McGraw-Hill Book Company, New York (1983) pp. 622–642
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© 1988 Springer Science+Business Media Dordrecht
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Greten, H., Beil, F.U. (1988). Lipase Deficiencies. In: Pollitt, R.J., Harkness, R.A., Addison, G.M. (eds) Studies in Inherited Metabolic Disease. Springer, Dordrecht. https://doi.org/10.1007/978-94-009-1259-5_3
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DOI: https://doi.org/10.1007/978-94-009-1259-5_3
Publisher Name: Springer, Dordrecht
Print ISBN: 978-94-010-7059-1
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