Centre for Neuromuscular Disease
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MRI is an ideal tool to study nerve lesions in patients and animals
Increased signal intensity of the common peroneal nerve (arrow) in a patient following nerve compression at the fibular head.
Hyperintense signal of the peroneal and tibial nerve (arrows) in the rat several days after transection of the sciatic nerve.
Animal Models
A strength of the centre is the breadth of expertise of PIs in the diverse aspects of neuromuscular disease. We will use this to forge internationally competitive translational research from bench to bedside and back. Research led by PIs in the proposed centre has identified pathogenic mutations in patients or rodent models of neuromuscular disease. These excellent individual research programs will be brought together into a unique array of coordinated research activities.
So far there is no consensus about the minimal standards that are required for the phenotypic analysis animal models of neuromuscular disease. In the past, the thrust of the analysis has more often been determined by the interest and available techniques of individual laboratories rather than by a rational complement of techniques. Moreover, there is no good understanding how readouts in animals relate to clinical endpoints. Often the tests that have traditionally been applied to animals are not easily transferable to patients.
Our aspiration is to establish the standard battery of tests for the comprehensive analysis of rodent models of neuromuscular disease. While many of the PIs have developed readout measures in their laboratories tailored to their individual needs, they have not brought together these results. The resources of the centre will be used to establish a communal framework under which the diverse manifestation of neuromuscular disease in rodents are examined. A particular focus will be to establish methods that closely relate the phenotypes of animals and humans.
We have established a core facility housing instruments for all participating laboratories which will permit a rapid analysis of animals. A unique feature of our group is that we can analysis the neurobiological basis of the behavioural manifestations of the disease in a coordinated way on a molecular, cellular and system level.
The figure illustrates the collective expertise of the researchers of the Centre in the analysis of rodents and its relationship to the clinical diagnosis and treatment of neuromuscular diseases (in blue).
(Click on picture for higher resultion image)
