Reduced efficiency of copper transport from cytosolic to noncytosolic fractions in LEC mutant rat.

Long-Evans Cinnamon (LEC) mutant rat, which spontaneously develops a necrotizing hepatic injury at 4-5 months of age, exhibits an excess hepatic copper accumulation. Neonatal LEC rat showed higher ratio of cytosolic to noncytosolic copper contents than neonatal normal rat, suggesting that the efficiency of copper transportation into noncytosolic compartment is primarily inherently reduced in LEC rat. Additionally using northern blot analysis with neonates we suggested that the elevated metallothionein levels observed in adult LEC rat are probably secondary to elevated copper levels.