| abstract |
Cybrid cell lines which have utility as model systems for the study of disorders that are associated with mitochondrial defects are described. The cybrids are constructed by treating immortal cell lines with an agent that irreversibly disables mitochondrial electron transport, and then transfecting the cells with mitochondria isolated from diseased tissue samples. Preferably, the immortal cell lines used are of an undifferentiated type that can be induced to differentiate, which results in the cybrids also being able to be induced to differentiate. One such cybrid was constructed using neuroblastoma cells and mitochondria from a patient suffering from Alzheimer's Disease. Methods for using such cybrids for screening drugs and therapies for utility in treating such disorders are also provided. In addition, cybrid animals, methods of producing them, and methods of using them in drug and therapy screening are also provided. |