Article
In vivo regulation of oxidative phosphorylation in cells harboring a stop-codon mutation in mitochondrial DNA-encoded cytochrome c oxidase subunit I.
The Journal of biological chemistry - 14 Dec 2001
D'Aurelio M, Pallotti F, Barrientos A, Gajewski C D, Kwong J Q, Bruno C, Beal M F, Manfredi G
Abstract excerpt
The mechanisms that regulate oxidative phosphorylation in mammalian cells are largely unknown. To address this issue, cybrids were generated by fusing osteosarcoma cells devoid of mitochondrial DNA (mtDNA) with platelets from a patient with a stop-codon mutation in cytochrome c oxidase subunit I (COX I). The molecular and biochemical characteristics of cybrids harboring varying levels of mutated mitochondrial DNA...
Topics
- Adenosine Triphosphate
- Blood Platelets
- Blotting, Northern
- Cell Line
- Codon, Terminator
- DNA Mutational Analysis
- DNA, Mitochondrial
- Electron Transport
- Electron Transport Complex IV
- Galactose
- Humans
- Hybrid Cells
- Immunoblotting