Skip to main content

Experimental Alkaptonuria in Animals

  • Chapter
  • 749 Accesses

Abstract

Throughout the history of research into alkaptonuria (AKU), there have been many attempts to develop animal models to study the disorder. Several authors have fed experimental animals with diets high in phenylalanine or tyrosine and detected homogentisic acid in the urine (Papageorge and Lewis 1938; Butts et al. 1938; Abbott and Salmon 1943). In some of these studies, it was reported that dietary phenylalanine was more effective than a comparative amount of tyrosine in inducing alkaptonuria, and this effect has never been completely resolved. Despite the elevated urinary levels of homogentisic acid achieved in these animals, no evidence for the deposition of ochronotic pigment in tissues was reported in any of these studies.

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

Buying options

Chapter
USD   29.95
Price excludes VAT (USA)
  • Available as PDF
  • Read on any device
  • Instant download
  • Own it forever
eBook
USD   39.99
Price excludes VAT (USA)
  • Available as EPUB and PDF
  • Read on any device
  • Instant download
  • Own it forever
Softcover Book
USD   54.99
Price excludes VAT (USA)
  • Compact, lightweight edition
  • Dispatched in 3 to 5 business days
  • Free shipping worldwide - see info
Hardcover Book
USD   54.99
Price excludes VAT (USA)
  • Durable hardcover 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

Learn about institutional subscriptions

References

  • Abbott, L.D., Salmon, C.L.: Experimental alkaptonuria in the white rat on high tyrosine diets. J. Biol. Chem. 150, 339 (1943)

    CAS  Google Scholar 

  • Butts, J.S., Dunn, M.S., Hallman, L.F.: Studies in amino acid metabolism. Metabolism of D/’-phenylalanine and Di-tyrosine in a normal rat. J. Biol. Chem. 123, 711–718 (1938)

    CAS  Google Scholar 

  • Montagutelli, X., Lalouette, A., Coudé, M., Kamoun, P., Forest, M., Guénet, J.L.: AKU, a mutation of the mouse homologous to human alkaptonuria, maps to chromosome 16. Genomics 19, 9–11 (1994)

    Article  CAS  PubMed  Google Scholar 

  • Moran, T.J., Yunis, E.J.: Studies on ochronosis. 2. Effects of injection of homogentisic acid and ochronotic pigment in experimental animals. Am. J. Pathol. 40, 359–369 (1962)

    PubMed Central  CAS  PubMed  Google Scholar 

  • Papageorge, E., Lewis, H.B.: Comparative studies of the metabolism of amino acids. VII. Experimental alcaptonuria in the white rat. J. Biol. Chem. 123, 211 (1938)

    CAS  Google Scholar 

  • Preston, A.J., Keenan, C.M., Sutherland, H., Wilson, P.J., Wlodarski, B., Taylor, A.M., Williams, D.P., Ranganath, L.R., Gallagher, J.A., Jarvis, J.C.: Ochronotic osteoarthropathy in a mouse model of alkaptonuria, and its inhibition by nitisinone. Ann. Rheum. Dis. 73, 284–289 (2014)

    Article  CAS  PubMed  Google Scholar 

  • Taylor, A.M., Preston, A.J., Paulk, N.K., Sutherland, H., Keenan, C.M., Wilson, P.J., Wlodarski, B., Grompe, M., Ranganath, L.R., Gallagher, J.A., Jarvis, J.C.: Ochronosis in a murine model of alkaptonuria is synonymous to that in the human condition. Osteoarthritis Cartilage 20, 880–886 (2012)

    Article  PubMed Central  CAS  PubMed  Google Scholar 

  • Tinti, L., Spreafico, A., Braconi, D., Millucci, L., Bernardini, G., Chellini, F., Cavallo, G., Selvi, E., Galeazzi, M., Marcolongo, R., Gallagher, I.A., Santucci, A.: Evaluation of antioxidant drugs for the treatment of ochronotic alkaptonuria in an in vitro human cell model. J. Cell. Physiol. 225, 84–91 (2010)

    Article  CAS  PubMed  Google Scholar 

Download references

Author information

Authors and Affiliations

Authors

Corresponding author

Correspondence to James A. Gallagher .

Editor information

Editors and Affiliations

Rights and permissions

Reprints and permissions

Copyright information

© 2015 Springer International Publishing Switzerland

About this chapter

Cite this chapter

Gallagher, J.A. (2015). Experimental Alkaptonuria in Animals. In: Rovenský, J., Urbánek, T., Oľga, B., Gallagher, J. (eds) Alkaptonuria and Ochronosis. Springer, Cham. https://doi.org/10.1007/978-3-319-15108-3_8

Download citation

  • DOI: https://doi.org/10.1007/978-3-319-15108-3_8

  • Publisher Name: Springer, Cham

  • Print ISBN: 978-3-319-15107-6

  • Online ISBN: 978-3-319-15108-3

  • eBook Packages: MedicineMedicine (R0)

Publish with us

Policies and ethics