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| Content Provider | World Health Organization (WHO)-Global Index Medicus |
|---|---|
| Author | Bundschuh, Freya A. Ludwig, Bernd Hoffmeier, Klaus |
| Description | Author Affiliation: Bundschuh FA ( Institut für Biochemie, Abteilung Molekulare Genetik, Johann Wolfgang Goethe-Universität, Frankfurt am Main, Germany. Freya@em.uni-frankfurt.de) |
| Abstract | Biogenesis of cytochrome c oxidase (COX) relies on a large number of assembly proteins, one of them being Surf1. In humans, the loss of Surf1 function is associated with Leigh syndrome, a fatal neurodegenerative disorder. In the soil bacterium Paracoccus denitrificans , homologous genes specifying Surf1 have been identified and located in two operons of terminal oxidases: surf1q is the last gene of the qox operon (coding for a $ba_{3}$ -type ubiquinol oxidase), and surf1c is found at the end of the cta operon (encoding subunits of the $aa_{3}$ -type cytochrome c oxidase). We introduced chromosomal single and double deletions for both surf1 genes, leading to significantly reduced oxidase activities in membrane. Our experiments on P. denitrificans surf1 single deletion strains show that both Surf1c and Surf1q are functional and act independently for the $aa_{3}$ -type cytochrome c oxidase and the $ba_{3}$ -type quinol oxidase, respectively. This is the first direct experimental evidence for the involvement of a Surf1 protein in the assembly of a quinol oxidase. Analyzing the heme content of purified cytochrome c oxidase, we conclude that Surf1, though not indispensable for oxidase assembly, is involved in an early step of cofactor insertion into subunit I. |
| ISSN | 00063002 |
| Journal | Biochimica et Biophysica Acta (BBA) - Reviews on Cancer |
| Issue Number | 10 |
| Volume Number | 1777 |
| Language | English |
| Publisher | Elsevier |
| Publisher Date | 2008-10-01 |
| Publisher Place | Netherlands |
| Access Restriction | Open |
| Subject Keyword | Bacterial Proteins Metabolism Electron Transport Complex IV Membrane Proteins Mitochondrial Proteins Paracoccus Denitrificans Protein Isoforms Amino Acid Sequence Genetics Isolation & Purification Mitochondria Enzymology Molecular Sequence Data Oxidation-Reduction Protein Subunits Sequence Alignment Research Support, Non-U.S. Gov't Biochemistry |
| Content Type | Text |
| Resource Type | Article |
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