OXPHOS Biogenesis


The research interests include the biogenesis of mitochondria and in particular the complexes involved in oxidative phosphorylation (OXPHOS), with a specific focus on one of the largest multi-protein complexes known in nature: Complex I (CI). The assembly of this giant enzyme is very intricate. It requires the combination of least 45 subunits which are encoded either by the nuclear or mitochondrial DNA.  This process requires a coordinated expression and targeting to the mitochondrial innermembrane and involves a number of yet unknown chaperone proteins. Defects in complex I assembly are the most frequently encountered OXPHOS disorders. Our translational research aims to improve diagnostics and develop new strategies for therapy.

Research questions investigated comprise: What is the sequence of assembly of subunits of CI? What are the crucial steps in the assembly? What are the functions of the individual accessory subunits of CI? How is the biogenesis of OXPHOS complexes regulated? How is assembly affected in CI deficient patients? Answers to these research questions provide insight in the molecular mechanisms leading to mitochondrial disorders.

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