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MITOCHONDRIAL BIOCHEMISTRYMitochondrial biochemistry The research topics addressed in the biochemistry group concern the functional characterization of novel defects of the mitochondrial energy generating system. The research focuses on two types of mitochondrial defects, namely on defects in the mitochondrial ATP production not due to a primary defect in one of the known mitochondrial enzymes, transporters and so on, and, secondly, on complex I deficiencies. The functioning of the mitochondrial ATP production machinery is examined in cell culture models of fibroblasts and primary muscle cell lines by ATP production and pyruvate oxidation rate measurements, respiratory chain enzyme activity assays, immunohistochemical and Western blot analysis of various mitochondrial enzymes. The second main interest is the identification of novel protein biomarkers for mitochondrial disorders. Using our large depository of patient-derived cell lines, we aim to identify proteins that are differentially expressed as a consequence of a complex I deficiency. For this purpose we make use of the state-of-the-art proteomics facility of the Radboud University Nijmegen Medical Centre, the Nijmegen Proteomics Centre. Contact Members
Gera Backx Frans van den Brandt Jacqueline van Haren-Uffing Karina Horsting-Wethly Merei Huigsloot, PhD Antoon Janssen, PhD Karen Janssen An Jonckheere, MD, MSc Antonia Kappen Theo van Lith Tesy Merkx Mina Pellegrini, PhD Özlem Seyrani Virginie van Steenveldt-Verkade Berendien Stoltenborg Thea van de Velden Diana Vermunt Hans Wessels Liesbeth Wintjes MITOCHONDRIAL BIOCHEMISTRY | ||