The chick embryo as a model system for ALS

Description:

What this research means to you: In order to learn more about the causes and mechanisms of MND, researchers rely heavily on the use of special cells  and animals that display the effects of the disease – these are known as laboratory “models”. This project will investigate the use of chick embryos as a rapid and inexpensive model of MND to determine the effects of genes that have been associated with MND, thus offering a valuable insight into disease mechanisms.

The researchers explain in more detail: In the coming years, a number of new genes that cause MND, modify the disease course or even confer protection are likely to be discovered. The biological relevance of these genes is investigated using different laboratory models, each with their own distinct advantages and disadvantages.

We have been developing a chick embryo system as a rapid and inexpensive means of determining the impact of potential new genes. Although it is not an adult model, it provides the advantages of a cell or tissue culture in simplicity, and the advantages of a genetically modified animal in being a whole organism.

Evidence from genetic studies suggests that certain variants of the genes VEGF and ELP3 might be protective against MND. During this project we intend to verify the effects of these genes in the chick embryo model of MND. By injecting VEGF and ELP3 genes alongside the mutant SOD1 gene, which is known to be damaging to motor neurones, we can investigate the ability of VEGF and ELP3 to “rescue” the neurones from harm. We will also use the model to study the effects of a recently discovered disease-causing gene called TDP-43. Our experiments will allow us to test the validity of the chick model, and key findings can be taken forward for examination in more complex (and expensive) animal models.