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Scientific scope
Neuropathology offers an integrative interface and plattform between clinical and basic neurosciences. Our research emphasis focus on diseases of the central nervous system. As many neurological diseases are still unsufficiently diagnosed and therapeutical options are limited, e. g. Alzheimer’s disease, Parkinson’s disease, apoplexia, tumors or multiple sclerosis, major issues will have to clarify underlying cellular and molecular pathomechanisms. Using animal experiments, cell culture models as well as surgical tissue specimens, the following research topics are addressed:
Focal Human Epilepsies and Epilepsy Database
Our major research topic focusses on therapy-refractory focal epilepsies in humans. To unravel the molecular pathogenesis of epilepsy-associated lesions, e.g. mesial temporal sclerosis, tumors and focal cortical dysplasias, large collections of histopathologically well characterized tissue specimens are available for molecular-biological, genetical as well as genomic analysis. Furthermore, extensive collaboration with our clinical and neuropathology colleagues from germany and many other european countries were helpful to establish the epilepsy database, a reference and consultation center for neurosurgical epilepsy tissue specimen.
Adult Stem Cells of the Human Brain
Numerous regions of the adult brain maintain precursor cells (“adult stem cells”), which are able to generate new neurons under physiological as well as pathophysiological conditions (i.e. neurogenesis). These intriguing precursor cells may gain outstanding importance in regenerative neurobiological treatment modalities and constitute an alternative approach to embryonic stem cells. Basic mechanisms of recruitment, proliferation and differentiation of adult stem cells remain, however, unknown. The identification of these mechanisms is a major topic of our project. The possibility to generate sufficient numbers of differentiated neurons or glia cells from autologues stem cells of the brain may unravel novel treatment strategies in apoplexia, Parkinson’s disease but also epilepsies or multiple sclerosis.
Neuro-Oncology
Neuro-oncology plays an important role in our clinico-neuropathological diagnostic efforts. With the international reputation of the neurosurgery department in Erlangen and its emphasis on the treatment of neuroendocrine tumors (e. g. pituitary adenomas, craniopharyngeomas) an unique collection of patients is available for a systematic molecular-neuropathological examination. Our research topics address three major questions:
- molecular tumorigenesis
- pathogenesis of brain invasion by tumor cells
- molecular genetic analysis as a tool for evaluating prognosis and therapy.
For our molecular-biological and genetical analysis we have access to a tissue bank comprising more than 500 unique tumors of the pituitary gland as well as 200 craniopharyngeomas (in collaboration with the neurosurgery department, Prof. Buchfelder, Prof. Schöffl).
Molecular Myopathology
A central research topic is the molecular characterisation of progressive skeletal muscle and cardiac diseases, which are morphologically characterized by pathological protein accumulation. These adult onset protein aggregate myopathies are clinically characterized by a progressive course leading to severe disability and premature death. To date, no causative treatment is available for these disorders. The main focus of our current research work (funded by the German Research Foundation, the Federal Ministry of Education and Research) is the generation and characterization of transgenic mouse models for the IBMPFD disease (Inclusion Body Myopathy associated with Pagets disease of bone and Fronototemporal Dementia) and the desmin myopathy and cardiomyopathy. The clinical, morphological, biochemical and molecular analysis of these mouse models shall provide deeper insights into offers the molecular “sequence” that leads to pathological protein aggregation and progressive muscle damage in these disorders. This work will be the basis for novel targeted treatment concepts.
