Cell transplantation in a transgenic model of Huntington’s disease

In this project we are investigating a potential aggravating effect of the Ccd42-interacting protein-4 (CIP-4) and a potential attenuating effect of the ciliary neurotrophic factor (CNTF) on the course of HD. Experiments will be performed using a recently established transgenic rat HD model, since it reflects largely the neuropathological and behavioural features of the human disease. CIP-4 and CNTF over-expressing neural progenitor cells (NPCs) will be transplanted into the striatum of transgenic HD rats at two time points, i.e. four weeks and three months post partum, respectively. 9 weeks, 6 months and 12 months later animals will be characterised with regard to survival, migration, differentiation and integration of the transplanted cells. Thereby, it will be investigated to which extent CIP-4 over-expressing NPCs influence the pathology of the disease and whether CNTF over-expressing NPCs are able to delay the onset of the disease and to reverse neuropathological features and clinical signs of the disease.