Kernicterus pp 51-64 | Cite as

The Gunn Rat Model

  • David W. McCandless
Part of the Contemporary Clinical Neuroscience book series (CCNE)


In 1938, an important paper appeared in The Journal of Heredity, which described for the first time a genetic mutation in Wistar rats resulting in neonatal jaundice (Gunn, C. 1938). The significance of this can hardly be overstated, as nearly all features of this murine model of hyperbilirubinemia correlate exactly with those of the human disorder known as the Crigler–Najjar syndrome. This has permitted extensive and meaningful data to be derived from animal studies. Because of this, knowledge of hyperbilirubinemia has been advanced at a rapid rate. This chapter will detail with some of the pertinent findings from the Gunn rat model.


Purkinje Cell Brain Mitochondrion Littermate Control Unconjugated Bilirubin Krebs Cycle Intermediate 
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  1. Batty, H., and Millhouse, O. (1976) Ultrastructure of the Gunn rat substantia nigra Acta Neuropathol. 34:7–19Google Scholar
  2. Billing, B. (1963) Bilirubin Metabolism. Postgrad Med. 39:176–182CrossRefGoogle Scholar
  3. Day, R. (1954) Inhibition of brain respiration in vitro by bilirubin: reversal of inhibition by various means. Am. J. Dis. Child. 88:504–510Google Scholar
  4. Farrell, D., et al. (1973) Feline GM3 gangliosidosis. J. Neuropath. Epxt. Neurol. 32:1–18CrossRefGoogle Scholar
  5. Gonatas, G., and Gonatas, J. (1965) Ultrastructural and biochemical observations on a case of systemic late infantile lipidoses. J. Neuropath. Exp. Neurol. 24:318–340PubMedCrossRefGoogle Scholar
  6. Hanefeld, F., and Natzschka, J. (1971) Histochemical studies in infant Gunn rayts with kernicterus. Neuropadiatrie 2:428–438PubMedCrossRefGoogle Scholar
  7. Johnson, L., et al. (1959) Kernicterus in rats with an inherited deficiency of glucuronyl transferase. Am. J. Dis. Child. 95:591–608Google Scholar
  8. McCandless, D., and Abel, M. (1980) The effect of unconjugated bilirubin on regional cerebellar energy metabolism. Neurobeh. Toxicol. 2:81–84Google Scholar
  9. Menken, M., Waggoner, J., and Berlin, N. (1966) The influence of bilirubin on oxidative phosphorylation and related reactions in brain and liver mitochondria: effect of protein binding. J. Neurochem. 13:1241–1248PubMedCrossRefGoogle Scholar
  10. Moore, P., and Karp, W. (1980) Further observations on the effect of bilirubin encephalopathy on the Purkinje cell population in Gunn rats. Epxt. Neurol. 69:408–413Google Scholar
  11. Nakata, D., Zakim, D., and Vessey, D. (1976) Defective function of a microsomal UDP-glucuronyltransferase in Gunn rats. Proc. Natl. Acad. Sci. 73:289–292PubMedCrossRefGoogle Scholar
  12. Rozdilsky, B. (1961) Experimental studies on the toxicity of bilirubin. In Kernicterus. Sass-Kortsak, A. ed. University of Toronto press, Toronto, ONGoogle Scholar
  13. Schenker, S., McCandless, D., and Zollman, P. (1966) Studies of Cellular toxicity of unconjugated bilirubin in kernicteric brain. J. Clin. Med. 45:1213–1220Google Scholar
  14. Schmid, R., et al. (1958) Congenital jaundice in rats due to a defect in glucuronide formation. J. Clin. Invest. 37:1123–1130PubMedCrossRefGoogle Scholar
  15. Schutta, H., Johnson, L., and Neville, H. (1970) Mitochondrial abnormalities in bilirubin encephalopathy. J. Neuropath. Exp. Neurol. 29:907–925Google Scholar
  16. Zetterstrom, R., and Ernster, L. (1956) Bilirubin, an uncoupler of oxidative phosphorylation in isolated mitochondria. Nature 178:1335–1337PubMedCrossRefGoogle Scholar
  17. Diamond, I., and Schmid, R. (1966b) Oxidative phosphorylation in experimental bilirubin encephalopathy. Science 155:1288–1289PubMedCrossRefGoogle Scholar
  18. Katoh, R., Kashiwamata, S., and Niwa, F. (1975) Studies on cellular toxicity of bilirubin: effect on the carbohydrate metabolism in the young rat brain. Brain Res. 83:81–90CrossRefGoogle Scholar
  19. Quastel, J., and Bickis, I. (1959) Metabolism of normal tissues and neoplasms in vitro. Nature 183:281–284PubMedCrossRefGoogle Scholar
  20. Gunn, C. 1938 Hereditary Acholuric Jaundice in a New Mutant Strain of Rats. J. Hered. 29:137–139Google Scholar
  21. Blanc, W., (1961) Kernicterus in Gunn’s strain of rats. In Kernicterus, Sass-Kortsak, A. ed. University of Toronto Press, Toronto, ON, pp 150–152Google Scholar
  22. Garray, E., Owen, C., and Flock, E. (1966) Formation of bilirubin in normal, damaged, and Gunn rat livers. J. Lab. Clin. Med. 67:817–829Google Scholar
  23. Odell, G., Natzschka, J., and Storey, G. (1967) Bilirubin nephropathy in the Gunn strain of rat. Am. J. Physiol. 212:931–938PubMedGoogle Scholar
  24. Hsia, D., et al. (1952) Erytyhroblastosis fetalis: studies of serum bilirubin in relation to kernicterus. N. Engl. J. Med. 247:668–673PubMedCrossRefGoogle Scholar
  25. Menken, M., and Weinbach, E. (1967) Oxidative phosphorylation and respiratory control of brain mitochondria isolated from kernicteric rats. J. Neurochem. 14:189–193PubMedCrossRefGoogle Scholar

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© Springer Science+Business Media, LLC 2011

Authors and Affiliations

  1. 1.Department of Cell Biology & AnatomyRosalind Franklin University of Medicine & Science, Chicago Medical SchoolNorth ChicagoUSA

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