Transmitochondrial Cytoplasmic Hybrid Cellular Model

Abstract: Transmitochondrial cytoplasmic hybrid (cybrid) cellular model is obtained by fusing r?cell line (host) completely devoid of mtDNA with cytoplasts produced by enucleation of cells (donor) derived from patients or controls. With the development of transmitochondrial technique， the methods of establishing cybrid cellular model are various. Now the model has been widely used not only in mitochondrial basic research but also in mitochondrial related diseases?clinical research. Cybrid cells can be studied in the context of a 憂eutral?nuclear background. Transmitochondrial cellular model is found extensive application in investigating mtDNA mutation and mitochondrial defect pathogenesis， evaluating the gene expression of mitochondrial genome at different level and screening the drug of treating mitochondrial diseases. Novel approaches such as those involving cytoplast fusion and mitochondrial microinjection are essential for gene therapy of diseases caused by these mutations， due to the non-Mendelian genetics of these diseases.

CSTR: 32200.14.cjcb.2006.01.0005