Regensburg 2010 – scientific programme

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BP: Fachverband Biologische Physik

BP 32: Posters: Physics of Cells

BP 32.11: Poster

Thursday, March 25, 2010, 17:15–20:00, Poster B1

Increase of cell stiffness in single muscle cells from patients with primary desminopathies — •Navid Bonakdar¹, Philip Kollmannsberger¹, Rolf Schröder², and Ben Fabry¹ — ¹Center for Medical Physics and Technology, Biophysics Group, Dept. of Physics, University of Erlangen-Nuremberg, Erlangen, Germany — ²Intitute of Neuropathology and Department of Neurology, University Hospital Erlangen, Germany

Desmin-related myopathies belong to the heterogenous group of distal-onset skeletal myopathies characterized by large accumulation of desmin (IFs) (Goebel et al. 1997), which compromises the ability of desmin to assemble into intermediate filaments (Sjoberg et al. 1999). Myofibrilar myopathies (MFMs) are histopathologically characterized by desmin-positive protein aggregates and myofibrillar degeneration and are caused by mutations in genes encoding for extramyofibrillar proteins. The disease usually develops in the second to third decade of life with signs of muscle weakness in the lower extremities and sometimes the heart. (Schröder et al., 2007). The precise molecular pathways and sequential steps that lead from an individual gene defect to progressive muscle damage are still unclear. (Schröder et al. 2009) Here we present the results of rheological measurements of myoblasts with and without desmin aggregation. Cell rheology is measured using FN-coated beads forced in a high-force Magnetic Tweezers setup. Stiffness of cells with desmin aggregation is markedly increased, indicating that desmin is directly involved with mechanical cell alteration that may contribute to the progression of MFMs.