Nephrotoxicity pp 633-638 | Cite as

Studies on a Physiological Model for the Hunman Renal Fanconi Syndrome

  • Karl S. Roth


The human renal Fanconi syndrome (FS) is unique among the many nephrotoxic disorders because of its association with a wide variety of inherited metabolic disorders as well as with numerous exogenous substances (1,2). This broad spectrum of known aetiologies lends credence to the concept that the renal tubular dysfunction of the FS represents the result of a common, underlying effect of a number of different agents on the kidney. Accordingly, investigators have utilized several animal models for the FS in efforts to elucidate the biochemical mechanism(s) implicit in this disorder. The most thoroughly-studied of these models is that generated by treatment of the rat with maleic acid, both in vivo (3, 4) and in vitro (58). To date, the specific biochemical action by which maleate affects transport of sugars, amino acids and various other endogenous materials remains elusive, although many intracellular and membrane transport phenomena have been demonstrated (5–10). However, the most significant disadvantage inherent in all studies using this compound is the fact that maleate is not an endogenously produced substance in mammals. Therefore, until very recently, no model system was available with which to study the FS associated with genetic disease in man.


Fanconi Syndrome Separate Determination Renal Tubular Dysfunction Automate Amino Acid Analyser Glycine Uptake 
These keywords were added by machine and not by the authors. This process is experimental and the keywords may be updated as the learning algorithm improves.


Unable to display preview. Download preview PDF.

Unable to display preview. Download preview PDF.


  1. 1.
    Roth, K.S. and Segal, S. In: Nephrology, J. Hamburger, J. Crosner and J.-P. Grunfeld, eds. , pp. 945–975. Wiley, NY 1979Google Scholar
  2. 2.
    Roth, K.S. , Foreman, J.W. and Segal, S. Kidney Int. 20:705, 1981PubMedCrossRefGoogle Scholar
  3. 3.
    Rosenberg, L.E. and Segal, S. Biochem. J. 92:345, 1964PubMedGoogle Scholar
  4. 4.
    Hergeron, M. and Vadeboncoeur, M. Nephron 8:367, 1971CrossRefGoogle Scholar
  5. 5.
    Roth, K.S. , Hwang, S.M. and Segal, S. Biochim Biophvs Acta 426: 675, 1976CrossRefGoogle Scholar
  6. 6.
    Roth, K.S. , Goldmann, D.R. and Segal, S. Pediat Res 12:1121, 1978PubMedCrossRefGoogle Scholar
  7. 7.
    Scharer, K. , Yoshida, T. , Voyer, L. , Berlow, S. , Pietra, G. and Metcoff, J. Res. Exp. Med. 157: 136, 1972CrossRefGoogle Scholar
  8. 8.
    Rogulski, J. , Pacanis, A. , Adamowicz, W. and Angielski, S. Acta Biochim Polon 21: 403, 1974Google Scholar
  9. 9.
    Pacanis, A. and Rogulski, J. Acta Biochim Polon 24:3, 1977PubMedGoogle Scholar
  10. 10.
    Szczepanska, M. and Angielski, S. Amer. J. Physiol. 239, F50, 1980Google Scholar
  11. 11.
    Lindblad, B. , Lindstedt, S. and Steen, A. Proc. Natl. Acad. Sci. USA 74: 4641, 1977PubMedCrossRefGoogle Scholar
  12. 12.
    Rosenberg, L.E. , Blair, A. and Segal, S. Biochim. Biophvs. Acta 54:479, 1961CrossRefGoogle Scholar
  13. 13.
    Booth, A.G. and Kenny, A.J. Biochem. J. 142:575, 1974PubMedGoogle Scholar
  14. 14.
    Weiss, S.D. , McNamara, P.D. , Pepe, L.M. and Segal, S. J. Membr. Biol. 43: 91, 1978PubMedCrossRefGoogle Scholar
  15. 15.
    Lowry, O.H. , Rosebrough, N.J. , Fan, A.L. and Randall, R.J. , J. Biol. Chem. 193:265, 1951.PubMedGoogle Scholar
  16. 16.
    McNamara, P.D. , Ozegovic, B. , Pepe, L.M. and Segal, S. Proc. Natl. Acad. Sci. USA 73: 4521, 1976PubMedCrossRefGoogle Scholar
  17. 17.
    Fallstrom, S.P. , Lindblad, B. and Steen, A. Acta Paediatr. Scand. 70:315, 1981PubMedCrossRefGoogle Scholar
  18. 18.
    Reynolds, R. , McNamara, P.D. and Segal, S. Life Sci 22:39, 1978.PubMedCrossRefGoogle Scholar

Copyright information

© Springer Science+Business Media New York 1989

Authors and Affiliations

  • Karl S. Roth
    • 1
  1. 1.Medical College of VirginiaVirginia Commonwealth UniversityMCV Station RichmondUSA

Personalised recommendations