Journal article
Intrinsic uncoupling of cytochrome c oxidase may cause the maternally inherited mitochondrial diseases MELAS and LHON
FEBS letters, v 433(1), pp 93-97
1998
PMID: 9738940
Featured in Collection : UN Sustainable Development Goals @ Drexel
Abstract
Mutations in the human mtDNA gene encoding subunit III of cytochrome
c oxidase (CO) have been reported to cause MELAS and LHON.
Paracoccus denitrificans cells expressing substitutions homologous to these MELAS- and LHON-causing mutations had lower growth yield than wild type cells and lower efficiency of proton pumping by CO (e.g. lower H
+/e
− ratio and lower ΔΨ), but had similar CO activity. These results indicate that both substitutions (F263L>A212T) cause intrinsic uncoupling, which may be the direct cause of the diseases. These results also suggest that subunit III is involved in proton pumping.
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Details
- Title
- Intrinsic uncoupling of cytochrome c oxidase may cause the maternally inherited mitochondrial diseases MELAS and LHON
- Creators
- Michael W Mather - Allegheny University of the Health SciencesHagai Rottenberg - Allegheny University of the Health Sciences
- Publication Details
- FEBS letters, v 433(1), pp 93-97
- Publisher
- Elsevier
- Resource Type
- Journal article
- Language
- English
- Academic Unit
- Microbiology and Immunology
- Web of Science ID
- WOS:000075577400021
- Scopus ID
- 2-s2.0-0031812281
- Other Identifier
- 991019168723304721
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InCites Highlights
Data related to this publication, from InCites Benchmarking & Analytics tool:
- Web of Science research areas
- Biochemistry & Molecular Biology
- Biophysics
- Cell Biology