Professor Agar researches the causes of ALS
by Reina Guerrero
Staff writer
News | 11/4/08

Professor Agar stands in front of his mass spectrometer, which he uses to analyze proteins. His research is aimed to discover potential causes of amyotrophic lateral sclerosis, known as as Lou Gehrig's Disease.
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Professor Agar (CHEM) and graduate student Qi Wang have been conducting research and have discovered potential causes of amyotrophic lateral sclerosis, which is commonly referred to as Lou Gehrig's disease.

Their findings were recently published in the Public Library of Science for Biology.

According to the Web site of the National Institute of Neurological Disorders and Stroke, ALS, a motor neuron disease, "is a rapidly progressive, invariably fatal neurological disease that attacks the nerve cells (neurons) responsible for controlling voluntary muscles." Motor neurons die and therefore are no longer able to send messages to muscles, which "gradually weaken, waste away and twitch. … Eventually the ability of the brain to start and control voluntary movement is lost. Individuals with ALS lose their strength and the ability to move their arms, legs and body. When muscles in the diaphragm and chest wall fail, individuals lose the ability to breathe without ventilatory support."

The disease was first described in 1869 by French neurologist Jean Martin Charcot. The common name for the disease is in honor of the baseball player who died of ALS in 1941. Once a person is diagnosed with Lou Gehrig's disease, the life expectancy ranges between two to five years.

However, 5 percent of the population affected by the disease will live for up to 20 years after being diagnosed, according to the ALS Association's Web site. Qi Wang said people who have Lou Gehrig's disease "gradually lose their abilities. They lose their ability to talk, swallow, and finally they lose the ability to breathe."

The exact causes of Lou Gehrig's disease are unknown; however, Agar explained that the research his lab is currently conducting is "trying to establish what causes the disease, which is unknown for 90 percent [of people affected by the disease]."

Agar wrote in an e-mail to the Justice, "Mutations in six different genes can cause ALS. Our group studies one such gene (which cause 20 percent of inherited ALS, or 2 percent of total)."

He further explained, "When we [the research assistants and Prof. Agar] find things that cause the disease we target them and try to make [them] stop happening." In an e-mail to the Justice, Professor Agar wrote, "We try to make 'rescue mutations,' which are second mutations that cancel the first (ALS-causing) mutations."

Agar said that he and Wang have "discovered some pretty important things about potential causes of Lou Gehrig's disease."

Agar explained that their research shows that "big clumps of protein" form.

Wang explained that the formation of clumps of protein is referred to as "protein aggregation." This refers to proteins sticking together, which does not happen to cells in a healthy body.

The stability of proteins is something else that contributes to the disease. Wang explained that protein stability involves protein mutation.

She said, "Normally, the proteins should have stability." She added, "We are trying to see what the changes of the mutation of the proteins are and how they correlate to the patient's survival."

"The only thing that we really care about at the end of the day is expanding life spans," Agar said.

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