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.


Sugar Filtration Cage Tyrosine Glycine 
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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

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