Absence of nigrostriatal degeneration in LEC rats up to 20 weeks of age.

摘要

BACKGROUND: Long-Evans Cinnamon (LEC) rat has a genetic defect of copper metabolism that is similar to human Wilson's disease. We studied the pathological changes in the nigrostriatal system of the LEC rat to examine the feasibility of using the LEC rat as a model of neurological Wilson's disease. METHODS: LEC and Long-Evans Agouti (LEA) rats were killed at 12 and 20 weeks of age. FluoroJade B staining and immunohistochemistry were performed and Western blot compared the amount of tyrosine hydroxylase (TH) protein. RESULTS: Degenerating neurons were not found in the substantia nigra (SN) and striatum. Dopaminergic neurons were of the same number in the SN of both LEC and LEA rats. Gliosis was of a similar degree in both animals. Western blot showed the same amount of TH protein in both animals. DISCUSSION: There was no evidence of neurodegeneration in the nigrostriatal system of the LEC rat up to developmental age 20 weeks. The LEC rat is not a suitable model for deposition of copper in the brain in human Wilson's disease.