The LEC Rat pp 133-141 | Cite as

Hereditary Low Levels of Plasma Ceruloplasmin in LEC Rats

  • Takao Ono
  • Syuiti Abe
  • Michihiro C. Yoshida


The LEC rats with hereditary hepatitis show an abnormal accumulation of copper in various organs such as liver, kidney, and brain, together with a marked decrease in the level of serum or plasma ceruloplasmin (Cp) [1]. Cp is a copper-binding protein primarily involved in the transport of copper from the liver [2]. The apoprotein is synthesized in the liver [3], and then binds from six to eight atoms of copper per Cp molecule [4, 5]. Most of the copper in plasma is present as the Cp-bound form. Thus, impaired copper metabolism may bear an etiological significance for the hepatic lesions in LEC rats, although the basic defect involved in the occurrence of hepatitis still remains obscure.


Hepatic Lesion Copper Level Copper Accumulation Plasma Ceruloplasmin Plasma Copper Concentration 
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.
    Li Y, Togashi Y, Sato S, Emoto T, Kang, J-H, Takeichi N, Kobayashi H, Kojima Y, Une Y, Uchino J (1991) Spontaneous hepatic copper accumulation in Long-Evans Cinnamon rats with hereditary hepatitis: A model of Wilson’s disease. J Clin Invest 87:1858–1861.PubMedCrossRefGoogle Scholar
  2. 2.
    Cousins RJ (1985) Absorption, transport, and hepatic metabolism of copper and zinc: Special reference to metallothionein and ceruloplasmin. Physiol Rev 65:238–309.PubMedGoogle Scholar
  3. 3.
    Ryden L, Bjork I (1976) Reinvestigation of some physicochemical and chemical properties of human ceruloplasmin (ferroxidase). Biochemistry 15:3411–3417.PubMedCrossRefGoogle Scholar
  4. 4.
    Koschinsky ML, Funk WD, van Oost BA, MacGillivray RTA (1986) Complete cDNA sequence of human preceruloplasmin. Proc Natl Acad Sci USA 83: 5086–5090.PubMedCrossRefGoogle Scholar
  5. 5.
    Schechinger T, Hartmann H-J, Weser U (1986) Copper transport from Cu(I)-thionein into apo-ceruloplasmin mediated by activated leucocytes. Biochem J 240:281–283.PubMedGoogle Scholar
  6. 6.
    Johnson DA, Osaki S, Frieden E (1967) A micromethod for the determination of ferroxidase (ceruloplasmin) in human serums. Clin Chem 13:142–150.PubMedGoogle Scholar
  7. 7.
    Ono T, Abe S, Yoshida MC (1991) Hereditary low level of plasma ceruloplasmin in LEC rats associated with spontaneous development of hepatitis and liver cancer. Jpn J Cancer Res 82:486–489.PubMedCrossRefGoogle Scholar
  8. 8.
    Masuda R, Yoshida MC, Sasaki M, Dempo K, Mori M (1988) Hereditary hepatitis of LEC rats is controlled by a single autosomal recessive gene. Lab Anim 22:166–169.PubMedCrossRefGoogle Scholar
  9. 9.
    Srai SKS, Burroughs AK, Wood B, Epstein O (1986) The ontogeny of liver copper metabolism in the guinea pig: Clues to the etiology of Wilson’s disease. Hepatology 6:427–432.PubMedCrossRefGoogle Scholar
  10. 10.
    Barrow L, Tanner MS, Critchley DR (1989) Expression of the caeruloplasmin gene in the adult and neonatal rat liver. Clin Sci 77:259–263.PubMedGoogle Scholar
  11. 11.
    Shokeir MHK (1971) Investigations on the nature of ceruloplasmin deficiency in the newborn. Clin Genet 2:223–227.PubMedCrossRefGoogle Scholar
  12. 12.
    Scheinberg IH, Gitlin D (1952) Deficiency of ceruloplasmin in patients with hepatolenticular degeneration (Wilson’s disease). Science 116:484–485.PubMedCrossRefGoogle Scholar
  13. 13.
    Czaja MJ, Weiner FR, Schwarzenberg SJ, Sternlieb I, Scheinberg IH, Van Thiel DH, LaRusso NF, Giambrone M-A, Krischner R, Koschinsky ML, MacGillivray RTA, Zern MA (1987) Molecular studies of ceruloplasmin deficiency in Wilson’s disease. J Clin Invest 80:1200–1204.PubMedCrossRefGoogle Scholar
  14. 14.
    Walshe JM (1975) The liver in hepatolenticular degeneration. In: Schiff L (ed) Diseases of the liver. J B Lippincott, Philadelphia, pp 1000–1016.Google Scholar
  15. 15.
    Nartey NO, Frei JV, Cherian MG (1978) Hepatic copper and metallothionein distribution in Wilson’s disease (hepatolenticular degeneration). Lab Invest 57:397–401.Google Scholar
  16. 16.
    Frydman M, Bonne-Tamir B, Farrer LA, Conneally PM, Magazanik A, Ashbel S, Goldwitch Z (1985) Assignment of the gene for Wilson’s disease to chromosome 13: Linkage to the esterase D locus. Proc Natl Acad Sci USA 82:1819–1821.PubMedCrossRefGoogle Scholar

Copyright information

© Springer-Verlag Tokyo 1991

Authors and Affiliations

  • Takao Ono
  • Syuiti Abe
  • Michihiro C. Yoshida
    • 1
  1. 1.Chromosome Research Unit, Faculty of ScienceHokkaido UniversitySapporo, 060Japan

Personalised recommendations