Synthesis and assembly of iron-sulphur (Fe/S) clusters into target proteins is a highly complex, coordinated and conserved process in living cells. Several machineries in both bacteria and eukaryotes have been discovered to assist Fe/S protein maturation. The importance of Fe/S proteins for human life and the comprehension at the molecular and cellular level of their biogenesis is documented by an increasing number of diseases linked to functional impairment of these proteins and of their maturation processes. Many of these diseases are fatal, sometimes in early childhood, which is not surprising based on the essential needs of Fe/S proteins in cellular metabolism. The founding member of Fe/S diseases is the neurodegenerative disorder Friedreich’s ataxia, in which the protein Frataxin is functionally deficient, either by a more than 70% decrease in protein levels as a result of decreased transcription or by point mutations. This functional impairment is associated with decreased activities of respiratory complexes I–III and of mitochondrial Aconitase
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The Biogenesis of Iron-sulfur Proteins: from Cellular Biology to Molecular Aspects
The main aim and objective of the Action is to address iron-sulfur (Fe/S) protein biogenesis in living systems and to investigate molecular mechanisms underlying human diseases related to Fe/S protein biogenesis dysfunctions. The Action will provide a molecular view of Fe/S proteins assembly processes and trafficking pathways at a systemic level, including their connections with cellular iron homeostasis processes.
Areas of expertise
Areas of Expertise relevant for the Action in the field of Biological Sciences: Systems Biology,General Biochemistry and Metabolism, Structural Biology (NMR, Crystallography, EM), Molecular biology and interactions, Biochemistry
Challenge
Iron-sulfur proteins represent one of the most extensively studied families of proteins. A growing interest in the field over the last decade has been observed; however, an integrated approach which provides a unified picture at system level is still missing. An inter- and multi-disciplinary background is therefore needed for the generation and training of creative, entrepreneurial and innovative scientists successfully engaged in the challenges of the modern research directed to impact in human health
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State-of-the-art
The past decade has led to the discovery of novel Fe/S proteins and insights into how their Fe/S cofactors are formed and incorporated into apoproteins.
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Aim of the Action
The Aim of the Action is to define a network able to coordinate and integrate intersectorial disciplines in Fe/S protein biogenesis.
Network
The challenge of this Action can be reached uniquely in the context of a pan European framework
COST meeting at Patras, Greece (Sept 2016)
Contact Information
Scientific Representative
Mario Piccioli
+390554575265
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Administrative Representative
Francesca Di Gloria
+390554574294
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Scientific Officer
Lucia Forzi
COST Association
Avenue Louise 149 1050 Brussels | Belgium
Administrative Officer
Svetlana Voinova
COST Association
Avenue Louise 149 1050 Brussels | Belgium
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