Mitochondrial genome distribution in histochemically cytochrome c oxidase-negative muscle fibres in patients with a mixture of deleted and wild type mitochondrial DNA
| Autor: | Steven J. Collins, Edward Byrne, C. Rudduck, S. Marzuki, Xenia Dennett |
|---|---|
| Rok vydání: | 1991 |
| Předmět: |
Mitochondrial DNA
Biopsy Molecular Sequence Data DNA Single-Stranded In situ hybridization Mitochondrion Genome DNA Mitochondrial Electron Transport Complex IV medicine Cytochrome c oxidase Humans Molecular Biology Ophthalmoplegia biology Base Sequence Histocytochemistry Cytochrome c Wild type Chromosome Mapping Nucleic Acid Hybridization Microtomy medicine.disease Molecular biology Mitochondria Muscle Mutation biology.protein Molecular Medicine Chronic progressive external ophthalmoplegia DNA Probes |
| Zdroj: | Biochimica et biophysica acta. 1097(4) |
| ISSN: | 0006-3002 |
| Popis: | In situ hybridization studies were performed on a series of chronic progressive external ophthalmoplegia patients harbouring large mitochondrial DNA deletions, using intra- and extra-deletional probes. Clear differences in the distribution of wild type and deleted mitochondrial genomes were seen in both ragged-red and non-ragged red, cytochrome c oxidase-negative fibres, with an accumulation of deleted genomes in the subsarcolemmal zone. Wild type genome content was normal or decreased in the cytochrome c oxidase-negative regions of one case, but in two patients, wild type mtDNA content in cytochrome c oxidase-negative regions was either normal (most fibres) or increased (occasional fibres). The latter observation suggests there may be a stage in the natural history of ragged-red fibre evolution where wild type genomes are transiently increased. The significance of this finding is discussed. |
| Databáze: | OpenAIRE |
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