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ALS/SMA — Miller

Michael Miller (ALS/SMA)
Miller and colleagues will use a nematode worm (Caenorhabditis elegans) model to test their central hypothesis. The nematode model system, which has motor neuron and muscle connections similar to those in humans, combines biochemical and genetic manipulation with the ability to directly monitor changes in mitochondria in live animals.
Amyotrophic Lateral Sclerosis (ALS)

MDA has awarded a research grant totaling $350,133 over three years to Michael Miller, associate professor in the department of biology at the University of Alabama, Birmingham. The grant will help support Miller's research into the fundamental molecular mechanisms responsible for the death of nerve cells called motor neurons in ALS (amyotrophic lateral sclerosis, or Lou Gehrig's disease) and spinal muscular atrophy (SMA).

Using nematode (worm) and fly research models, Miller and colleagues have generated preliminary data suggesting that mutations in genes associated with familial (inherited) ALS and SMA disrupt a signaling mechanism that controls function of the cellular "energy factories" called mitochondria, which are necessary for motor neuron health.

Now, using a nematode model, the study team plans to combine biochemical and genetic manipulation with the ability to directly monitor changes in the worm’s cellular mitochondria as a means of uncovering the fundamental molecular mechanisms responsible for motor neuron death in ALS and SMA — mechanisms the team hypothesizes may influence mitochondrial function.

Miller expects the major impacts of his work will be the identification of mitochondria as the linchpin in neurodegenerative disorders including ALS and SMA, and the long list of drug targets his team expects to identify through its screening process.

"Funding by the MDA for this project is critically important," Miller said, "as without this support, the momentum gained over the past few years would come to a halt."

Funding for this MDA grant began February 1, 2011.

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