DELETION OF THE WILSONS-DISEASE GENE IN HEREDITARY HEPATITIS LEC RATS

LEC rats develop disorder of copper metabolism and hepatitis similar t o those of human Wilson's disease. We recently demonstrated that the g ene responsible for hepatitis (hts) of LEC rats is homologous to Wilso n's disease gene (WD). The present study showed a deletion of at least 90 base pair of WD cDNA in LEC rats, which corresponds to nucleotides 3981 to 4071 in human WD cDNA sequence. This deletion was linked with hepatic copper accumulation and hepatitis, and considered to be a pri mary mutation for hepatic disorder in the LEC rat. The WD gene was ass igned to rat chromosome 16 at band q12.2-q12.4 by fluorescence in situ hybridization (FISH).