Significance of Respirasomes for the Assembly/Stability of Human Respiratory Chain Complex I
We showed that the human respiratory chain is organized in supramolecular assemblies of respiratory chain complexes, the respirasomes. The mitochondrial complexes I (NADH dehydrogenase) and III (cytochrome c reductase) form a stable core respirasome to which complex IV (cytochrome c oxidase) can als...
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Veröffentlicht in: | The Journal of biological chemistry 2004-08, Vol.279 (35), p.36349-36353 |
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Sprache: | eng |
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Zusammenfassung: | We showed that the human respiratory chain is organized in supramolecular assemblies of respiratory chain complexes, the respirasomes.
The mitochondrial complexes I (NADH dehydrogenase) and III (cytochrome c reductase) form a stable core respirasome to which complex IV (cytochrome c oxidase) can also bind. An analysis of the state of respirasomes in patients with an isolated deficiency of single complexes
provided evidence that the formation of respirasomes is essential for the assembly/stability of complex I, the major entry
point of respiratory chain substrates. Genetic alterations leading to a loss of complex III prevented respirasome formation
and led to the secondary loss of complex I. Therefore, primary complex III assembly deficiencies presented as combined complex
III/I defects. This dependence of complex I assembly/stability on respirasome formation has important implications for the
diagnosis of mitochondrial respiratory chain disorders. |
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ISSN: | 0021-9258 1083-351X |
DOI: | 10.1074/jbc.M404033200 |