Objective Defects in the mitochondria! oxidative phosphorylation (OXPHOS-) system, the principal circuit for cellular energy production, lead to often fatal, multi-system disorders affecting organs and tissues with a high-energy demand. The EUMITOCOMBAT consortium, consisting of 12 partners, encompassing 21 scientific groups from nine different countries, including the Czech Republic, aims to integrate and extend knowledge on basic aspects of OXPHOS biology and the pathobiological cascades underlying OXPHOS disease manifestation in humans.The EUMITOCOMBAT project will re-inforce Europe as the international leader in the development of treatments capable of combating these disorders. To this end we will:- Integrate clinical and model-system expertise.- Characterize important genes and proteins involved in the formation and regulation of the OXPHOS-system.- Study the network of components involved in cellular energy metabolism by functional genomics.- Develop more efficient genetic and protein (mutation) screening methods.- Evaluate the cellular alterations/adaptations of these defects in specified cells, tissues, models and humans.- Develop methods to target and test therapeutic agents to specific (sub)cellular locations. Fields of science medical and health scienceshealth sciencespublic healthnatural sciencesbiological sciencesgeneticsDNAnatural sciencesbiological sciencesgeneticsnucleotidesnatural scienceschemical scienceselectrochemistryelectrophoresisnatural scienceschemical sciencesanalytical chemistrymass spectrometry Keywords disease energy errors inborn inborn errors of energy metabolism metabolism metabolism orphan of orphan orphan disease Programme(s) FP6-LIFESCIHEALTH - Life sciences, genomics and biotechnology for health: Thematic Priority 1 under the Focusing and Integrating Community Research programme 2002-2006. Topic(s) LSH-2002-2.1.1-4 - Rare disorders of mitochondria or of nuclear organisation with broader implications for biological processes Call for proposal FP6-2002-LIFESCIHEALTH See other projects for this call Funding Scheme IP - Integrated Project