A marked increase in free copper levels in the plasma and liver of LEC rats: An animal model for Wilson disease and liver cancer

Free Radical Research

Volumn 28, Issue 5, 1998, Pages 441-450

  Koizumi, Masahiko ^a   Fujii, Junichi ^a   Suzuki, Keiichiro ^a   Inoue, Takehiro ^a   Inoue, Toshihiko ^a   Gutteridge, John M C ^a   Taniguchi, Naoyuki ^a  

^a OSAKA UNIVERSITY MEDICAL SCHOOL   (Japan)

Author keywords

Bleomycin detectable iron; Fenton chemistry; Hepatitis; LEC rats; Phenanthroline detectable copper; Reactive oxygen species

Indexed keywords

CERULOPLASMIN; COPPER; FREE RADICAL; HYDROXYL RADICAL; IRON;

ACUTE HEPATITIS; ANIMAL EXPERIMENT; ANIMAL MODEL; ANIMAL TISSUE; ARTICLE; BLOOD LEVEL; CONTROLLED STUDY; ENZYME ACTIVITY; IRON BLOOD LEVEL; LIVER CANCER; LIVER CELL CARCINOMA; LIVER DYSFUNCTION; LIVER LEVEL; METAL BINDING; NONHUMAN; OXIDATION REDUCTION STATE; PROTEIN BINDING; RAT; WILSON DISEASE;

ANIMALIA; CINNAMOMUM VERUM;

EID: 0031857603     PISSN: 10715762     EISSN: None     Source Type: Journal    
DOI: 10.3109/10715769809066881     Document Type: Article

References (36)

1. Bull, P.C., Thomas, G.R., Rommens, J.M., Forbes, J.R. and Cox, D.W. (1993). The Wilson disease gene is a putative copper transporting P-type ATPase similar to the Menkes gene. Nature Genetics, 5, 327-337.
2. Petrukhin, K., Fischer, S.G., Pirastu, M., Tanzi, R.E., Chernov, I., Devoto, M., Brzustowicz, L.M., Cayanis, E., Vitale, E., Russo, J.J., Matseoane, D., Boukhgalter, B., Wasco, W., Figus, A.L., Loudianos, J., Cao, A., Sternlieb, I., Evgrafov, O., Parano, E., Pavone, L., Warburton, D., Ott, J., Penchaszadeh, G.K., Scheinberg, I.H. and Gilliam, T.C. (1993). Mapping, cloning and genetic characterization of the region containing the Wilson disease gene. Nature Genetics, 5, 338-343.
3. Tanzi, R.E., Petrukhin, K., Chernov, I., Pellequer, J.L., Wasco, W., Ross, B., Romano, D.M., Parano, E., Pavone, L., Brzustowicz, L.M., Devoto, M., Peppercorn, J., Bush, A.I., Sternlieb, I., Pirastu, M., Gusella, J.F., Evgrafov, O., Penchaszadeh, G.K., Honig, B., Edelman, I.S., Soares, M. B., Scheinberg, I.H. and Gilliam, T.C. (1993). The Wilson disease gene is a copper transporting ATPase with homology to the Menkes disease gene. Nature Genetics, 5, 344-350.
4. Li, Y., Togashi, Y., Sato, S., Emoto, T., Kang, J. -H., Takeichi, N., Kobayashi, H., Kojima, Y., Une, Y. and Uchino, J. (1991). Abnormal copper accumulation in non-cancerous and cancerous liver tissues of LEC rats developing hereditary hepatitis and spontaneous hepatoma. Japanese Journal of Cancer Research, 82, 490-492.
5. Li, Y., Togashi, Y., Sato, S., Emoto, T., Kang, J. -H., Takeichi, N., Kobayashi, H., Kojima, Y., Une, Y. and Uchino, J. (1991). Spontaneous hepatic copper accumulation in Long-Evans Cinnamon rats with hereditary hepatitis. Journal of Clinical Investigations, 87, 1858-1861.
6. Miyoshi, E., Fujii, J., Hayashi, N. and Taniguchi, N. (1997). LEC rats: an overview of recent findings. Journal of Trace Elements in Experimental Medicine, 10, 135-145.
7. Mori, M., Yoshida, M.C., Takeichi, N. and Taniguchi, N. (1991). The LEC Rat: a New Model for Hepatitis and Liver Cancer. Springer-Verlag, Tokyo.
8. Wu, J., Forbes, J.R., Chen, H.S. and Cox, D.W. (1994). The LEC rat has a deletion in the copper transporting ATPase gene homologous to the Wilson disease gene. Nature Genetics, 7, 541-545.
9. Yamaguchi, Y., Heiny, M.E., Shimizu, N., Aoki, T. and Gitlin, J.D. (1994). Expression of the Wilson disease gene is deficient in the Long-Evans Cinnamon rat. Biochemical Journal, 301, 1-4.
10. Murata, Y., Yamakawa, E., Iizuka, T., Kodama, H., Abe, T., Seki, Y. and Kodama, M. (1995). Failure of copper incorporation into ceruloplasmin in the Golgi apparatus of LEC rat hepatocytes. Biochemical and Biophysical Research Communications, 209, 349-355.
11. Okayasu, T., Tochimaru, H., Hyuga, T., Takahashi, T., Takekoshi, Y., Li, Y., Togashi, Y., Takeichi, N., Kasai, N. and Arashima, S. (1992). Inherited copper toxicity in Long-Evans Cinnamon rats exhibiting spontaneous hepatitis: a model of Wilson's disease. Pediatric Research, 31, 253-257.
12. Evans, P.J., Bomford, A. and Halliwell, B. (1989). Non-caeruloplasmin copper and ferroxidase activity in mammalian serum. Ferroxidase activity and phenanthroline-detectable copper in human serum in Wilson's disease. Free Radical Research Communications, 7, 55-62.
13. Ogihara, H., Ogihara, T., Miki, M., Yasuda, H. and Mino, M. (1995). Plasma copper and antioxidant status in Wilson's disease. Pediatric Research, 37, 219-226.
14. Gutteridge, J.M.C., Winyard, P.G., Blake, D.R., Lunec, J., Brailsford, S. and Halliwell, B. (1985). The behavior of caeruloplasmin in stored human extracellular fluids in relation to ferroxidase II activity, lipid peroxidation and phenanthroline detectable copper. Biochemical Journal, 230, 517-523.
15. Gutteridge, J.M.C., Cao, W. and Chevion, M. (1990). Bleomycin-detectable iron in brain tissue. Free Radical Research Communications, 11, 317-320.
16. Gutteridge, J.M.C. (1984). Copper-phenanthroline-induced site-specific oxygen-radical damage to DNA. Detection of loosely bound trace copper in biological fluids. Biochemical Journal, 218, 983-985.
17. Gutteridge, J.M.C., Rowley, D.A. and Halliwell, B. (1981). Superoxide-dependent formation of hydroxyl radicals in the presence of iron salts. Detection of 'free' iron in biological systems by using bleomycin-dependent degradation of DNA. Biochemical Journal, 199, 263-265.
18. Gutteridge, J.M.C. and Hou, Y. (1986). Iron complexes and their reactivity in the bleomycin assay for radical-promoting loosely-bound iron. Free Radical Research Communications, 2, 143-151.
19. Gutteridge, J.M.C., West, M., Eneft, K. and Floyd, R.A. (1990). Bleomycin-iron damage to DNA with formation of 8-hydroxydeoxyguanosine and base propenals. Indications that xanthine oxidase generates superoxide from DNA degradation products. Free Radical Research Communications, 10, 159-165.
20. Johnson, D.A., Osaki, S. and Frieden, E. (1967). A micromethod for the determination of ferroxidase (cerulo-plasmin) activity in human serums. Clinical Chemistry, 13, 142-150.
21. Harris, Z.L., Takahashi, Y., Miyajima, H., Serizawa, M., MacGillivray, R.T.A. and Gitlin, J.D. (1995). Aceruloplasminemia: molecular characterization of this disorder of iron metabolism. Proceedings of the National Academy of Sciences of the USA, 92, 2539-2543.
22. Suzuki, K.T., Yamamoto, K., Kanno, S., Aoki, Y. and Takeichi, N. (1993). Selective removal of copper bound to metallothionein in the liver of LEC rats by tetrathiomolybdate. Toxicology, 83, 149-158.
23. Sugawara, N., Katakura, M., Li, D., Sugawara, C. and Miyake, H. (1993). Role of hepatic copper-metallothionein on liver function of Long-Evans Cinnamon rats with a new mutation causing hereditary hepatitis. Research Communications in Chemical Pathological and Pharmacology, 83, 349-358.
24. Sawaki, M., Enomoto, K., Hattori, A., Tsuzuki, N., Sugawara, N. and Mori, M. (1994). Role of copper accumulation and metallothionein induction in spontaneous liver cancer development in LEC rats. Carcinogenesis, 15, 1833-1837.
25. Sakurai, H., Satoh, H., Hatanaka, A., Sawada, T., Kawano, K., Hagino, T. and Nakajima, K. (1994). Unusual generation of hydroxyl radicals in hepatic copper-metallothionein of LEC (Long-Evans Cinnamon) rats in the presence of hydrogen peroxide. Biochemical and Biophysical Research Communications, 199, 313-318.
26. Sugawara, N., Sato, M., Yuasa, M. and Sugawara, C. (1995). Biliary excretion of copper, metallothionein and glutathione into Long-Evans Cinnamon rats: a convincing animal model for Wilson disease. Biochemical and Molecular Medicine, 55, 38-42.
27. Suzuki, K.T. (1995). Disordered copper metabolism in LEC rats, an animal model of Wilson disease: roles of metallothionein. Research Communications in Molecular Pathology and Pharmacology, 89, 221-240.
28. Ogra, Y., Ohmichi, M. and Suzuki, K.T. (1996). Mechanisms of selective copper removal by tetrathimolybdate from metallothionein in LEC rats. Toxicology, 106, 75-83.
29. Suzuki, K., Miyazawa, N., Nakata, T., Seo, H.G., Sugiyama, T. and Taniguchi, N. (1993). High copper and iron levels and expression of Mn-superoxide dismutase in mutant rats displaying hereditary hepatitis and hepatoma (LEC rats). Carcinogenesis, 14, 1881-1884.
30. Gutteridge, J.M.C. and Halliwell, B. (1989). Iron toxicity and oxygen radicals. In Clinical Haematology: Iron Chelating Therapy (C. Hershko, ed.), Bailliere Tindall, London, pp. 195-256.
31. Halliwell, B., Gutteridge, J.M.C. and Cross, C.E. (1992). Free radicals, antioxidants and human diseases: where are we now? Journal of Laboratory and Clinical Medicine, 6, 598-620.
32. Grootveld, M., Bell, J.D., Halliwell, B., Aruoma, O.I., Bonford, A. and Sadles, P. (1989). Non-transferrin-bound iron in plasma or serum from patients with idiopathic haemochromatosis. Characterization by high performance liquid chromatography and nuclear magnetic resonance spectroscopy. Journal of Biological Chemistry, 264, 4417-4421.
33. Yamada, T., Sogawa, K., Suzuki, Y., Izumi, K., Agui, T. and Matsumoto, K. (1992). Elevation of the level of lipid peroxidation associated with hepatic injury in LEC mutant rat. Research Communications in Chemical Pathology and Pharmacology, 77, 121-124.
34. Kato, J., Kohgo, Y., Sugawara, N., Katsuki, S., Sintani, N., Fujikawa, K., Miyazaki, E., Kobune, M., Takeichi, N. and Niitsu, Y. (1993). Abnormal hepatic iron accumulation in LEC rats. Japanese Journal of Cancer Research, 84, 219-222.
35. Kato, J., Kobune, M., Kohgo, Y., Sugawara, N., Hisai, H., Nakamura, T., Sakamaki, S., Sawada, N. and Niitsu, Y. (1996). Hepatic iron deprivation prevents spontaneous development of fulminant hepatitis and liver cancer in Long-Evans Cinnamon rats. Journal of Clinical Investigations, 98, 923-929.
36. Hagen, T.M., Huang, S., Curnutte, J., Fowler, P., Martinez, V., Wehr, C.M., Ames, B.N. and Chisari, F.V. (1994). Extensive oxidative DNA damage in hepatocytes of transgenic mice with chronic active hepatitis destined to develop hepatocellular carcinoma. Proceedings of the National Academy of Sciences of the USA, 91, 12808-12812.