Abstract
A rhesus monkey model for familial hypercholesterolemia was established. The affected animals were heterozygous for a nonsense mutation of the LDL-receptor gene in a position 284 of exon 6 and presented with high plasma levels of total cholesterol, LDL cholesterol and apo B. Plasma concentration of Lp(a) did not correlate with LDL-R deficiency but rather with apo(a) phenotype. The results support the concept that the metabolism of Lp(a) is independent of that of LDL.
This is a preview of subscription content, log in via an institution to check access.
Access this chapter
Tax calculation will be finalised at checkout
Purchases are for personal use only
Preview
Unable to display preview. Download preview PDF.
Similar content being viewed by others
References
Watanabe, Y. 1980. Serial inbreeding of rabbits with hereditary hyper- lipidemia (WHHL rabbit) - Incidence and development of atherosclerosis and xanthoma. Atherosclerosis 36: 261–268.
Russell, D. W. Lehrman, M.A., Sudhof, T.C., Yanamt, T, Davis, C.G, Hobbs H.H., Brown, M.S. and Goldstein, J.L. 1986. The LDL receptor in familial hypercholesterolemia: use of human mutations to dissect a membrane protein. Cold Spring Harbor Symp. Quantit. Biology, 51: 811–819.
Scanu, A.M., Khalil, A., Neven, L.G., Tidore, M., Dawson, G., Pfaffinger, D, Jackson, E., Carey, K.D., McGill, H.C., and Fless, G.M. 1988. Genetically determined hypercholesterolemia in a rhesus monkey family due to a deficiency of the LDL receptor. J. Lipid Res. 29: 1671–1681.
Neven, L.G., Khalil, Al, Pfaffinger, D., Jackson, E., and Scanu, A.M. 1990. Rhesus monkey model of familial hypercholesterolemia: relation between plasma Lp(a) levels, apo(a) isoforms and LDL receptor function. J. Lipid Res. In Press.
Hummel, M., Zhigao Li, Neven, L.G., and Scanu, A.M. 1988. Definition of the molecular basis of the LDL receptor deficiency in a rhesus monkey pedigree with familial hypercholesterolemia. Proc. Natl. Aca. Sci., USA. In Preparation.
Scanu, A. 1988. Lipoprotein(a): A genetically determined lipoprotein containing a glycoprotein of the plasminogen family. Sem. in Thromb. and Hemost. 14: 266–270.
Utermann, G., Menzel, H.J., Kraft, H.G., Duba, C., Kemmler, H.G. and Seitz, C. 1987. Lipoprotein(a) glycoprotein phenotype inheritance and relation to Lp(a) concentration in plasma. J. Clin. Invest. 80: 458–465.
Brewer H.B., 1990. Effectiveness of Diet and Drugs in the treatment of patients with elevated Lp(a) levels. In Lipoprotein(a): 25 years of progress. Scanu, A.M. Editor. Acad. Press, N.Y. In press.
Author information
Authors and Affiliations
Editor information
Editors and Affiliations
Rights and permissions
Copyright information
© 1990 Kluwer Academic Publishers
About this chapter
Cite this chapter
Scanu, A.M. (1990). Lipoprotein(a) and the LDL receptor (LDL-R): examination of the problem in a pedigree of rhesus monkeys with a familial hypercholesterolemia secondary to LDL-R deficiency. In: Descovich, G., Gaddi, A., Magri, G., Lenzi, S. (eds) Atherosclerosis and Cardiovascular Disease. Springer, Dordrecht. https://doi.org/10.1007/978-94-009-0731-7_15
Download citation
DOI: https://doi.org/10.1007/978-94-009-0731-7_15
Publisher Name: Springer, Dordrecht
Print ISBN: 978-94-010-6814-7
Online ISBN: 978-94-009-0731-7
eBook Packages: Springer Book Archive