کد مقاله کد نشریه سال انتشار مقاله انگلیسی نسخه تمام متن
1942865 1537048 2010 7 صفحه PDF دانلود رایگان
عنوان انگلیسی مقاله ISI
The electron transfer flavoprotein: Ubiquinone oxidoreductases
موضوعات مرتبط
علوم زیستی و بیوفناوری علوم کشاورزی و بیولوژیک دانش گیاه شناسی
پیش نمایش صفحه اول مقاله
The electron transfer flavoprotein: Ubiquinone oxidoreductases
چکیده انگلیسی

Electron transfer flavoprotein:ubiqionone oxidoreductase (ETF-QO) is a component of the mitochondrial respiratory chain that together with electron transfer flavoprotein (ETF) forms a short pathway that transfers electrons from 11 different mitochondrial flavoprotein dehydrogenases to the ubiquinone pool. The X-ray structure of the pig liver enzyme has been solved in the presence and absence of a bound ubiquinone. This structure reveals ETF-QO to be a monotopic membrane protein with the cofactors, FAD and a [4Fe–4S]+ 1+2 cluster, organised to suggests that it is the flavin that serves as the immediate reductant of ubiquinone. ETF-QO is very highly conserved in evolution and the recombinant enzyme from the bacterium Rhodobacter sphaeroides has allowed the mutational analysis of a number of residues that the structure suggested are involved in modulating the reduction potential of the cofactors. These experiments, together with the spectroscopic measurement of the distances between the cofactors in solution have confirmed the intramolecular pathway of electron transfer from ETF to ubiquinone. This approach can be extended as the R. sphaeroides ETF-QO provides a template for investigating the mechanistic consequences of single amino acid substitutions of conserved residues that are associated with a mild and late onset variant of the metabolic disease multiple acyl-CoA dehydrogenase deficiency (MADD).

Research Highlights
► ETF:QO is an iron-sulfur flavoprotein linking central metabolism with the respiratory chain.
► Structural and mutagenesis studies suggest that FAD is the electron donor to ubiquinone (UQ).
► Mutations in the UQ binding domain can cause a mild lipid storage myopathy that is riboflavin responsive.

ناشر
Database: Elsevier - ScienceDirect (ساینس دایرکت)
Journal: Biochimica et Biophysica Acta (BBA) - Bioenergetics - Volume 1797, Issue 12, December 2010, Pages 1910–1916
نویسندگان
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