Neurologische Klinik und Poliklinik
Josef-Schneider-Straße 11, 97080 Würzburg
Research foci (and basic equipment-based research projects):
The main focus of the group is to understand pathomechanisms in inherited neuropathies
with the aim to develop treatment strategies for the still uncurable disorders.
An important tool for our research are spontaneous and engineered mouse mutants that mimic the human disorders with respect to genetic defects and pathological alterations. In this context, an important observation was that in some mutants, immune
cells (T-lymphocytes, macrophages) are found in the diseased nerves. By cross-breeding the
animal models with mutants with compromised immune cell function, we could demonstrate that the immune cells significantly contribute to the pathological alterations in the nerves. Future studies are underway with the aim to elucidate the role of the immune cell in the pathogenesis of inherited nerve disorders in detail.
In a second approach we try to prevent or to reduce the loss of distal portions of axons in dys- and demyelinating models. Axonal loss has profound clinical consequences for patients, such as progressive weakness and muscle atrophy. In a first step, we demonstrated that in mice with a severe dysmyelinating neuropathy approximately 75% of the axon terminals of long peripheral nerves degenerate within a few months. Experiments using neurotrophic factors are now underway trying to prevent this axonal loss .
Another topic deals with a particular form of acquired neuropathy, the Miller Fisher syndrome (MFS). We could show by immunoelectron microscopy that antibodies from MFS patients bind to pre- and postsynaptic membranes of neuromuscular junctions of mouse hemidiaphragms, a finding that supports previous electrophysiological studies using patch-clamp technology (Dr. Buchwald, Prof. Toyka).