One way to understand the function of a gene and how this goes wrong in disease is to create a model in mice. These projects aim to develop new and better models of MND to understand the causes of MND. A selection of our newest model projects are highlighted below.
Download our research we fund information sheet for details of all of the projects we fund.
Creating a Zebrafish C9ORF72 model of MND
- Dr Andrew Grierson and Dr Kurt De Vos
- University of Sheffield
- £86,687 (Biomedical project) over three years
- Start date: October 2013
- Our ref: 6292 Oct12
This project aims to understand the function of the C9ORF72 gene, and to determine how defects in this gene lead to the development of MND.The first aim is to create a zebrafish model for studying C9ORF72. They will do this by removing or blocking the C9ORF72 gene and determining whether the zebrafish develop MND. This will allow the research group to learn about the role of C9ORF72 in the brain and spinal cord, and to determine the effect of defective C9ORF72 in the context of an animal model.
The second aim is based on some new evidence from recent experiments in Sheffield that C9ORF72 regulates the way that cells are able to break down damaged components. This process is called autophagy. This project will directly test whether C9ORF72 defects associated with MND lead to disruption of autophagy in the developed zebrafish model.
Development of a new C9ORF72 mouse and cellular model using BAC technology
- Dr Javier Alegre Abarrategui
- University of Oxford
- £240,906 (Biomedical project) over three years
- Start date: January 2013
- Our Ref: 6087 Oct 12
Previous work at Oxford, with MND Association funding, looked at the development of mouse models with the TDP-43 and FUS genes. In this project Dr. Alegre Abarrategui will look at using genetic technology to develop a new mouse model of the C9ORF72 gene that can be ‘switched’ on and off. He will also use the same technology to develop a new cellular model by inserting the C9ORF72 gene into human motor neurone cell lines.
The investigation of different mouse models for causative changes in MND
- Prof Linda Greensmith
- University College London
- £242,135 (Biomedical project) over three years
- Start date: October 2012
- Our Ref: 6086 Oct 12
No single mouse model reflects all the characteristics of MND. In this project two recently developed mouse models (along with data from the well-established SOD1 model) will be investigated to compare common pathways of cell death and dysfunction in motor neurones. The team want to specifically identify likely causative changes that happen very early on in the disease.
