mitoMORPH:
State of the art
Mitochondria are essential, dynamic and complex organelles that play crucial roles in many aspects of cellular function. They supply the cell with ATP generated by oxidative phosphorylation. Additionally, mitochondria are the site of numerous metabolic pathways, play a key role in the assembly of Fe/S-clusters and are central regulators of programmed cell death. To maintain this multitude of different functions mitochondria behave as dynamic organelles. Their location and shape within the cell are controlled by the concerted action of the fusion and fission machineries of the organelle. Because mitochondria perform so many different functions in different tissues, mitochondrial defects are often tightly coupled with a variety of human diseases.
Aims of the project
The subproject aims to understand the link between abnormalities in mitochondrial morphology and function and the pathogenic mechanism in mitochondrial diseases. The first goal is to establish standardized assays for the assessment of mitochondrial shape as a morphological parameter, mitochondrial membrane potential and oxygen consumption as tools for mitochondrial function, and steady-state levels of mitochondrial proteins and the lipid composition of mitochondrial membranes as biochemical markers. In fibroblasts and myoblasts from patients with mitochondriopathies and controls we want to correlate the disease with morphological and functional alterations. The combined application of all these assays will allow us to use these parameters as biomarkers for the diagnosis of various diseases and for the effectiveness of applied medical treatments. Such comprehensive assays collection can provide novel and unique possibility for monitoring the disease stage of various mitochondriopathies. Finally, we provide this platform for mitochondrial morphology and function analyses to the other members of the MitoNET.
Project management and contact addresses
Ludger Schöls, Professor
Department of Neurology and Hertie Institute for Clinical Brain Research
University of Tübingen
Hoppe-Seyler-Str. 3, 72076 Tübingen
Tel: +49-7071-2980412
Fax: +49-7071-294839
E-mail: Ludger.Schoels@uni-tuebingen.de
Doron Rapaport, Professor
Interfaculty Institute for Biochemistry
University of Tübingen
Hoppe-Seyler-Str. 4, 72076 Tübingen
Tel: +49-7071-2974184
Fax: +49-7071-295070
E-mail: Doron.Rapaport@uni-tuebingen.de
