MECHANISMS OF EXPRESSION OF DELETED MITOCHONDRIAL DNA FROM CULTURE CELLS

点击翻译按钮获取中文摘要

Our method developed for cryopreservation and subsequent culture of muscle, skin and lymphocytes could accomplish the maintenance of abnormal mitochondria DNA and the cellular phenotypic characteristics in vitro. The cultured cells including muscle, skin fibro-blast and lymphocytes were more than two thousand samples from two hundred patients suspected with mitochondrail abnormalities by the clinical diagnosis. These cells were used for the analysis of the gene expression of abnormal mitochodrial DNA. We then attempted to develop the software for database about the information of these cells for future use.2. Mutant mitochondrial DNA with large-scale deletions have been frequently observed in patients with chronic progressive external opthalmolplegia(CPEO), a sub group of the mitochondrial encephalomyopathies. To exclude involvement of the nuclear genome in expression of the mitochondrial dysfunction characteristic of CPEO, we introduced the mtDNA of CPEO patient into clonal mtDNA-less HeLa cells, and isolated cybrid clones.3. We attempted to establish cell lines without mitochodria by inactivating mitochondrial DNA with the treatment of ethidium bromide to investigate whether the observed abnormality comes from nuclear genes of mitochondrial genes.