Article
Expression of the Wilson disease gene is deficient in the Long-Evans Cinnamon rat.
The Biochemical journal - 1 Jul 1994
Yamaguchi Y, Heiny M E, Shimizu N, Aoki T, Gitlin J D
Abstract excerpt
Long-Evans Cinnamon rats develop a necrotizing hepatitis characterized by excessive hepatic copper accumulation, defective holoceruloplasmin biosynthesis and impaired biliary copper excretion. To elucidate the molecular basis of this defect, a cDNA clone encoding the rat Wilson disease gene was isolated and used to examine gene expression in selected tissues from normal and Long-Evans Cinnamon rats. Although this...
Topics
- Adenosine Triphosphatases
- Amino Acid Sequence
- Animals
- Base Sequence
- Cation Transport Proteins
- Copper
- Copper-Transporting ATPases
- DNA, Complementary
- Gene Expression
- Hepatolenticular Degeneration
- Humans
