Prof. Dr. Roland Lill (Marburg): Biogenesis of iron-sulfur proteins in eukaryotes and associated diseases |
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| Startdatum/-zeit: | 20.05.2010 16:15 |
| Enddatum/-zeit: | 20.05.2010 |
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| Adresse: | Prof. Dr. Christian Ungermann |
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| Homepage: | http://www.biologie.uni-osnabrueck.de/Fachbereich/?x=ae,expl,aaig |
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| Adresse: | Barbarastr. 11, Hörsaal 35/E01 FB Biologie/Chemie, Barbarastr. 11 49076 Osnabrück |
| Karte: | Bei Google Maps anzeigen |
| Beschreibung: | Research over the past few years has established the crucial role of mitochondria in both the biogenesis of iron-sulfur (Fe/S) proteins and the regulation of cellular iron homeostasis. The two processes are coupled mechanistically in that defects in Fe/S protein biogenesis elicit increases in both cellular and mitochondrial iron import. The molecular basis of Fe/S cluster synthesis and assembly into apoproteins in a living cell has been subject to intense research activities (see Reviews). Biogenesis is accomplished by three complex proteinaceous machineries (Figure). Mitochondrial Fe/S proteins require the iron-sulfur cluster (ISC) assembly machinery which was inherited from bacteria during evolution. Cytosolic/nuclear Fe/S protein assembly also requires the function of this machinery, yet additionally depends on the mitochondrial ISC export apparatus and the cytosolic iron-sulfur protein assembly (CIA) machinery. The components of all three systems (more than 25 proteins) are highly conserved in eukaryotes from yeast to man suggesting similar mechanisms of Fe/S protein assembly. We are currently attempting to define the molecular basis of the assembly processes, in particular the contribution of mitochondria to cytosolic/nuclear Fe/S protein biogenesis. In fact, the latter process is the major reason why mitochondria (or mitochondria-derived organelles such as mitosomes and hydrogenosomes) are indispensable in virtually all eukaryotic cells. Essential cytosolic/nuclear Fe/S proteins depending on mitochondrial ISC function are involved in DNA replication and repair as well as ribosome assembly and translation, thus linking the function of mitochondria to basic processes of gene expression in a eukaryotic cell. The process is of importance for human disease in that more than ten diseases are associated with ISC, CIA or Fe/S protein defects. For instance, depletion of the ISC assembly component frataxin leads to the neurodegenerative disease Friedreich’s ataxia, and a defect in the ISC export protein ABCB7 is associated with X-linked sideroblastic anemia and ataxia (XLSA/A). Fe/S protein defects in the nucleus impairing DNA replication and repair link Fe/S protein biogenesis to numerous diseases including various forms of cancer. The seminar will provide an introduction into the mechanisms underlying Fe/S protein biogenesis in eukaryotes and summarize the phenotypical consequences upon defects. |
| Kategorie: | Biologisches Kolloqium |
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