Neuro facility teams with UK firm to develop MND model

A commercial alliance has been formed between the National Neuroscience Facility (NNF) and UK company Danio Labs, to develop a zebrafish model for Motor Neurone Disease (MND) and other neurodegenerative diseases.

The Howard Florey Institute has already established a breeding facility for zebrafish. The alliance with Danio will allow access to the company's zebrafish transgenesis and high-throughput screening platforms, allowing HFI researchers to be trained in the techniques. The company will also assist with commercialisation of drugs developed by the researchers.

According to Howard Florey Institute researcher Assoc Prof Surindar Cheema, there are several hundred mutations known to be responsible for the development of MND, a debilitating and rapid neurodegenerative disorder that affects the spinal cord, brain stem and neocortex. Patients suffer from progressive limb paralysis, loss of speech, and inability to swallow and ultimately difficulty in breathing, which leads to death within 3-5 years.

While mouse models can and have been used to study MND, Cheema says there are too many mutations to develop mouse models for high-throughput screening cost-effectively. However, the zebrafish offers a rapid and low-cost alternative with the capacity for high-throughput screening for drug development.

About half of the inherited form of the disease can be associated with mutations in three genes, Sod-1, Alsin gene or the spinal muscular atrophy (SMA) gene. But around 90 per cent of MND cases are sporadic, indicating that environmental risk factors may also play a role. Zebrafish may also play a useful role in determining some of these factors, says Cheema.

The transgenic zebrafish will be examined in a number of different ways, to see whether proteins involved in the development of the disease are over-expressed, and whether the characteristic neurodegeneration occurs. In addition, some mutations may cause behavioural modifications, such as a slowdown in movement.

Cheema says that while the initial focus of the Zebrafish program will be targeting MND, the technology will be applicable to other neurodegenerative diseases including Alzheimer's and Parkinson's diseases.