Skip to main content

Disorders of Cholesterol Metabolism

Cholesterol Storage Diseases

  • Chapter
Disorders of Lipid Metabolism
  • 182 Accesses

Abstract

Cholesterol is a white waxy sterol lipid that occurs in all animal cells. It has an important structural role in cell membranes, is the precursor for steroid hormones in the adrenal gland, and is the precursor for bile acids in the liver. Because of its insolubility in water, it is solubilized and transported in the blood as a lipoprotein complex. Cholesterol input to the body comes from the diet and endogenous synthesis, primarily by the liver. Cholesterol output occurs via secretion in the bile, conversion to bile acids, and loss from sloughing off of cells from the skin and intestines; a very small amount is lost in the urine. Lactating females also lose some cholesterol during breast feeding. The daily balance of cholesterol metabolism in humans is shown in Fig. 4–1.

This is a preview of subscription content, log in via an institution to check access.

Access this chapter

Chapter
USD 29.95
Price excludes VAT (USA)
  • Available as PDF
  • Read on any device
  • Instant download
  • Own it forever
eBook
USD 84.99
Price excludes VAT (USA)
  • Available as EPUB and PDF
  • Read on any device
  • Instant download
  • Own it forever
Softcover Book
USD 109.99
Price excludes VAT (USA)
  • Compact, lightweight edition
  • Dispatched in 3 to 5 business days
  • Free shipping worldwide - see info

Tax calculation will be finalised at checkout

Purchases are for personal use only

Institutional subscriptions

Preview

Unable to display preview. Download preview PDF.

Unable to display preview. Download preview PDF.

References

  • Ahlberg, J., Angelin, B., Bjorkhem, I., Einarsson, K., and Leijd, B., 1979, Hepatic cholesterol metabolism in normo- and hyperlipidemic patients with cholesterol gallstones, J. Lipid Res., 20:107.

    PubMed  CAS  Google Scholar 

  • Ahlberg, J., Angelin, B., and Einarsson, K., 1981, Hepatic 3-hydroxy-3-methylglutaryl coenzyme A reductase activity and biliary lipid composition in man: relation to cholesterol gallstone disease and effects of cholic acid and chenodeoxycholic acid treatment, J. Lipid Res., 22:410.

    PubMed  CAS  Google Scholar 

  • Angelin, B., Ewerth, S., and Einarsson, K., 1983, Ursodeoxycholic acid treatment in cholesterol gallstone disease: effects on hepatic 3-hydroxy-3-methylglutaryl coenzyme A reductase activity, biliary lipid composition, and plasma lipid levels, J. Lipid Res., 24:461.

    PubMed  CAS  Google Scholar 

  • Carey, M. C., 1978, Critical tables for calculating the cholesterol saturation in native bile, J. Lipid Res., 19:945.

    PubMed  CAS  Google Scholar 

  • Carey, M. C., and Small, D. M., 1978, The physical chemistry of cholesterol solubility in bile, J. Clin. Invest., 61:998.

    Article  PubMed  CAS  Google Scholar 

  • Devlin, T. M., 1982, Textbook of Biochemistry with Clinical Correlations, John Wiley & Sons, New York.

    Google Scholar 

  • Groen, A. K., Ottenhoff, R., Jansen, P. L. M., van Marie, J., and Tytgat, G. N. J., 1989, Effect of cholesterol nucleation-promoting activity on cholesterol solubilization in model bile, J. Lipid Res., 30:51.

    PubMed  CAS  Google Scholar 

  • Gutierrez, C., Okita, R., and Krisans, S., 1988, Demonstration of cytochrome reductases in rat liver peroxisomes: biochemical and immunochemical analyses, J. Lipid Res., 29:613.

    PubMed  CAS  Google Scholar 

  • Murray, R. K., Granner, D. K., Mayes, P. A., and Rodwell, V. W., 1988, Harper’s Biochemistry, 21st ed., Appleton & Lange, Norwalk, Connecticut.

    Google Scholar 

  • Poznansky, M. J., Hutchison, S. K., and Davis, P. J., 1989, Enzyme replacement therapy in fibroblasts from a patient with cholesteryl ester storage disease, FASEB J., 3:152.

    PubMed  CAS  Google Scholar 

  • Reynier, M. O., Montet, J. C., Gerolami, A., Marteau, C., Crotte, C., Montet, A. M., and Mathieu, S., 1981, Comparative effects of cholic, chenodeoxycholic, and ursodeoxycholic acids on micellar solubilization and intestinal absorption of cholesterol, J. Lipid Res., 22:467.

    PubMed  CAS  Google Scholar 

  • Sabine, J.R., 1977, Cholesterol, Marcel Dekker, New York.

    Google Scholar 

  • Salvioli, G., Igimi, H., and Carey, M. C., 1983, Cholesterol gallstone dissolution in bile. Dissolution kinetics of crystalline cholesterol monohydrate by conjugated chenodeoxycholate-lecithin and conjugated ursodeoxycholate-lecithin mixtures: dissimilar phase equilibria and dissolution mechanisms. J. Lipid Res., 24:701.

    PubMed  CAS  Google Scholar 

  • Smith, B. F., 1987, Human gallbladder mucin binds biliary lipids and promotes cholesterol crystal nucleation in model bile, J. Lipid Res., 28:1088.

    PubMed  CAS  Google Scholar 

  • Smith, E. L., Hill, R. L., Lehman, I. R., Lefkowitz, R. J., Handler, P., and White, A., 1983a, Principles of Biochemistry: Mammalian Biochemistry, 7th ed., McGraw-Hill Book Co., New York.

    Google Scholar 

  • Smith, E. L., Hill, R. L., Lehman, I. R., Lefkowitz, R. J., Handler, P., and White, A., 1983, Principles of Biochemistry: General Aspects, 7th ed., McGraw-Hill Book Co., New York.

    Google Scholar 

  • Stryer, L., 1988, Biochemistry, W. H. Freeman & Co., San Francisco.

    Google Scholar 

  • Suckling, K. E., and Stange, E. F., 1985, Role of acyl-CoA: cholesterol acyltransferase in cellular cholesterol metabolism, J. Lipid Res., 26:647.

    PubMed  CAS  Google Scholar 

Download references

Author information

Authors and Affiliations

Authors

Rights and permissions

Reprints and permissions

Copyright information

© 1990 Plenum Press, New York

About this chapter

Cite this chapter

Marinetti, G.V. (1990). Disorders of Cholesterol Metabolism. In: Disorders of Lipid Metabolism. Springer, Boston, MA. https://doi.org/10.1007/978-1-4615-9564-9_5

Download citation

  • DOI: https://doi.org/10.1007/978-1-4615-9564-9_5

  • Publisher Name: Springer, Boston, MA

  • Print ISBN: 978-1-4615-9566-3

  • Online ISBN: 978-1-4615-9564-9

  • eBook Packages: Springer Book Archive

Publish with us

Policies and ethics