Purkinje cell transplants in Shaker mutant rats with hereditary Purkinje cell degeneration and ataxia.

Shaker mutant rats are characterized by the adult-onset degeneration of cerebellar anterior lobe Purkinje cells and temporally correlated development of ataxia and tremor. Normal E-13 Purkinje cells were transplanted into the anterior cerebellum in adult shaker mutant rats to study donor/host interactions in an animal with adult-onset heredodegeneration. Donor Purkinje cells from extraparenchymal transplant sites migrated radially into the host molecular layer and differentiated. Donor Purkinje cell dendrites expanded to fill the host molecular layer, spinous processes were apparent, and axonal projections into the host gray and white matter were observed. Donor Purkinje cells remaining in the extraparenchymal transplant sites differentiated if they were located relatively close to the host cerebellum. Donor Purkinje cells located intraparenchymally in the host white matter or granule cell layer survived, but were stunted in their development. The orthogonal movement of donor Purkinje cells away from transplant sites in the host cerebellum was spatially restricted. The findings from this study indicate that host cerebellar cortex with adult-onset heredodegeneration of Purkinje cells supports the survival and differentiation of transplanted normal embryonic Purkinje cells.