Consortium
A consortium of six research groups from across
Europe
with long-standing expertise in techniques and relevant research fields performs a multi-faceted approach for the study of the molecular mechanisms underlying HSP. The Partners represent a background ranging over clinical medicine, clinical biochemistry, medical genetics, and basic research in the fields of protein biochemistry and neurobiology. The research centres involved are well-established institutions that can guarantee to provide the infrastructure necessary for a successful outcome of the project. The consortium, furthermore, is closely linked to clinical institutions and physicians concerned with familial spastic paraplegia that have expressed their interest in sharing patient samples and clinical data as well as in implementing for validation any novel diagnostic or therapeutic tools evolving from the project. The project is divided into 3 principal components to provide a comprehensive approach to investigate the process of motor neuron degeneration. WP1 involves the production of 7 novel mouse models for HSP and their extensive characterisation, in parallel with 1 model recently developed and characterised by one of the partners. WP2 is geared towards a detailed analysis of mitochondrial function and mitochondrial-based protein degradation, whilst WP3 is intended to provide basic information about the function of three molecules mutated in HSP, and to examine the role of defective transportation in the pathogenesis of this disease.
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