ALS Association - Wisconsin Chapter: The ALS Association Funds Development of New Model System to Study ALS

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The ALS Association Funds Development of New Model System to Study ALS

ALSA Initiates Project to Create SOD1 Zebrafish for Screening Therapeutics

The ALS Association (ALSA) today announced funding of a new effort to create a model of ALS in a laboratory animal of growing popularity among neurobiologists, the zebrafish. The project award is to investigators at Ohio State University in Columbus, Christine Beattie, Ph.D., and Arthur Burghes, Ph.D. With the new model system, Drs. Beattie and Burghes intend to provide an excellent new tool for investigating ALS therapies.

“ALSA is very excited to have two outstanding established investigators focusing their efforts on ALS research,” said ALSA’s Science Director Lucie Bruijn, Ph.D. “The development of a zebra fish model will be an invaluable tool to identify new targets and screen potential compounds for ALS.”

The co-investigators will be creating fish with an alteration in the gene coding for superoxide dismutase (SOD1). This is the gene altered to produce some of the inherited forms of ALS in people. Many different mutations in SOD1 can produce the disease. The project will investigate three particular SOD1 mutations. Drs. Burghes and Beattie will also see if other genes play a part in expression of the SOD1 mutations. “Once identified, these modifiers can also serve as targets for drug screens,” the investigators said.

Genetic mosaics will also be created. These fish will have only some cells with the mutation. Work in rodents revealed recently that surrounding healthy cells can save cells that bear the SOD1 mutation.

The investigators said that making mosaic zebrafish “will allow us to address what cells contribute to the disease, and the effect of mutant cells on wild-type motor neurons.”

The project represents “an approach that also has the potential to reveal disease mechanisms,” the investigators said, “these experiments will characterize the disease process at the cellular level both before and after overt defects occur.”

Zebrafish are increasingly popular among developmental and neurobiologists, as the species has amenable genetics, the basic vertebrate genome is present, and the larvae are transparent. The ALSA funded investigators will use a marker gene to follow how neurons bearing the mutation grow, extend, and form connections. “For a disease as complex as ALS,” the investigators said, “generation of different animal model systems is essential as each model organism offers its own advantages.”