Animal models

A strength of the centre is the breadth of expertise of Principal Investigators (PIs) in the diverse aspects of neuromuscular disease.

We use this to strengthen internationally competitive translational research.

Research led by centre PIs has identified pathogenic mutations in patients or rodent models of neuromuscular disease. These excellent individual research programmes will be brought together into a unique collection of coordinated research activities.

Challenges

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, analysis has more often been determined by the interest and available techniques of individual laboratories rather than by available techniques.

There is also a lack of understanding of how readouts in animals relate to humans.

Communal Frameworks

Our aim is to establish the standard set 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 are enabling the establishment of a communal framework under which neuromuscular diseases in rodents are examined.

A particular focus will be to establish methods that closely relate the phenotypes of animals and humans.

Facilities

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 analyse the neurobiology of different diseases in a coordinated way on a molecular, cellular and system level.