Skip to main content

Regulation of Heme Biosynthesis in Hepatic and Erythroid Cells: A Brief Introduction to the Pathobiochemistry of Human Porphyrias

  • Conference paper
Diagnosis and Therapy of Porphyrias and Lead Intoxication

Abstract

Disturbances of heme biosynthesis differ from most other metabolic diseases in that they involve a pathway essential to life. Heme (Feprotoporphyrin IX) functions as a prosthetic group for hemoglobin, myoglobin, mitochondrial and microsomal cytochromes, catalase, peroxidases, and tryptophan pyrrolase. Important physiologic functions of heme are transport of oxygen, activation of oxygen, and the transfer of electrons to oxygen. All cells with aerobic metabolism are presumed to be able to synthesize heme.

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 119.00
Price excludes VAT (USA)
  • Available as EPUB and PDF
  • Read on any device
  • Instant download
  • Own it forever
Softcover Book
USD 159.99
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

  1. Condie, L.W., Tephly, T.R., Baron, J.: Studies on heme synthesis in the rat adrenal. Ann. Clin. Res. 8 (Supp. 17), 83 (1976)

    CAS  Google Scholar 

  2. DeGoeij, A.F.P.M., Christianse, K., van Steveninck, J.: Decreased heme synthetase activity in blood cells of patients with erythropoietic protoporphyria. Eur. J. Clin. Invest. 5, 379 (1975)

    Google Scholar 

  3. Granick, S., Sassa, S.: δ-Aminolevulinic acid synthetase and the control of heme and chlorophyll synthesis. In: Metabolic Regulation. Vogel, H.J. (ed.). New York-London: Academic Press 1971, p. 77

    Google Scholar 

  4. Meyer, U.A.: Intermittent acute porphyria: Clinical arid biochemical studies of disordered heme biosynthesis. Enzyme 16, 334 (1973)

    PubMed  CAS  Google Scholar 

  5. Meyer, U.A., Schmid, R.: The porphyrias. In: The Metabolic Basis of Inherited Disease. Stanburg, J.B., Wyngaarden, J.B., Fredrickson, D.S. (eds.). New York: McGraw-Hill 1978, p. 1166

    Google Scholar 

  6. Neuwirt, J., Ponka, P., Borova, J.: The role of heme in the regulation of δ-aminolevulinic acid and heme synthesis in rabbit reticulocytes. Eur. J. Biochem. 9, 36 (1969)

    Article  PubMed  CAS  Google Scholar 

  7. Sardesai, V.M., Waldmann, J., Orten, J.M.: A comparative study of porphyrin biosynthesis in different tissues. Blood 24, 178 (1964)

    PubMed  CAS  Google Scholar 

  8. Sassa, S., Solish, G., Levere, R.D., Kappas, A.: Studies in porphyria. IV. Expression of the gene defect of acute intermittent porphyria in cultured human skin fibroblasts and amniotic cells: Prenatal diagnosis of the porphyric trait. J. Exp. Med. 142, 722 (1975)

    Article  PubMed  CAS  Google Scholar 

  9. Sassa, S.: Sequential induction of heme pathway enzymes during erythroid differentiation of mouse friend leukemia virus-infected cells. J. Exp. Med. 143, 305 (1976)

    Article  PubMed  CAS  Google Scholar 

  10. Sassa, S., Zalar, G.L., Kappas, A.: Deficient induction of uroporphyrinogen-I synthetase activity in mitogen-stimulated lymphocytes from patients with acute intermittent porphyria. Clin. Res. 25, 517A (1977)

    Google Scholar 

  11. Schmid, R., Schwartz, S., Watson, C.J.: Porphyrin content of bone marrow and liver in various forms of porphyria. Arch. Intern. Med. 93, 167 (1954)

    CAS  Google Scholar 

  12. Schwartz, S., Stephenson, B., Sarkar, D., Freyholtz, H., Runge, W.: Development and implications of experimental renal porphyria. In: Porphyrins in Human Diseases. Doss, M. (ed.). Basel: Karger 1976, p. 370

    Google Scholar 

  13. Strand, L.J., Felscher, B.W., Redeker, A.G., Marver, H.S.: Enzymatic abnormalities in heme biosynthesis in intermittent acute porphyria. Decreased hepatic conversion of porphobilinogen to porphyrins and increased δ-aminolevulinic acid synthetase activity. Proc. Natl. Acad. Sci. U.S.A. 67, 1315 (1970)

    Article  PubMed  CAS  Google Scholar 

  14. Whiting, M.J.: Synthesis of δ-aminolevulinate synthase by isolated liver polyribosomes. Biochem. J. 158, 391 (1976)

    PubMed  CAS  Google Scholar 

Download references

Author information

Authors and Affiliations

Authors

Editor information

Editors and Affiliations

Rights and permissions

Reprints and permissions

Copyright information

© 1978 Springer-Verlag Berlin Heidelberg

About this paper

Cite this paper

Meyer, U.A. (1978). Regulation of Heme Biosynthesis in Hepatic and Erythroid Cells: A Brief Introduction to the Pathobiochemistry of Human Porphyrias. In: Doss, M. (eds) Diagnosis and Therapy of Porphyrias and Lead Intoxication. Springer, Berlin, Heidelberg. https://doi.org/10.1007/978-3-642-67002-2_1

Download citation

  • DOI: https://doi.org/10.1007/978-3-642-67002-2_1

  • Publisher Name: Springer, Berlin, Heidelberg

  • Print ISBN: 978-3-540-08863-9

  • Online ISBN: 978-3-642-67002-2

  • eBook Packages: Springer Book Archive

Publish with us

Policies and ethics