2008 Woodie Awards
Possible progress on Gehrig's disease
Zhi Xiong - The Daily Iowan
Issue date: 1/29/08 Section: Metro
A diagnosis of amyotrophic lateral sclerosis, nicknamed after the baseball legend Lou Gehrig, is a slow death sentence.
The disease kills by gradually paralyzing its victims. Motor neurons in the brain and spinal cord die, cutting off communication pathways to the muscles they control. Eventually, even the muscles necessary for swallowing and breathing atrophy and die.
There is still no cure for the fatal disease, which affects 30,000 Americans, according to the National Institutes of Health.
But research has yielded promising findings - last week UI scientists published their study of a drug that doubled the average life spans of mice with the inherited form of the disorder. The report was published online last week in the Journal of Clinical Investigation.
"It transfers to the quality of life," said lead scientist John Engelhardt, a UI professor and the head of the anatomy/cell-biology department. "There's potential that the drug could be helpful."
The scientists discovered a protein interaction that leads to overproduction of a molecule that is damaging in high quantities and linked to neurodegenerative diseases.
The protein complex works like a furnace with a broken thermostat that produces too much heat, Engelhardt said. A drug called apocynin hinders production of the culprit molecule by disabling one component.
In an earlier study, Engelhardt and his team worked directly with genes to double the life span of mice. Apocynin, which can be extracted from plants and easily made in labs, also achieved such effects when fed to the mice through their water.
"It's turning off the 'furnace' by a different method," he said.
The disease usually strikes between age 40 and 60, and it is more common in men than women. Approximately half of people diagnosed with the illness live more than three years, and only 10 percent more than 10 years.
After the Jan. 24 study was published, Engelhardt found himself poring over the disease's Therapy Development Institute's website, where patients read published research and comment on the blog. The study has already generated hundreds of posts.
The disease kills by gradually paralyzing its victims. Motor neurons in the brain and spinal cord die, cutting off communication pathways to the muscles they control. Eventually, even the muscles necessary for swallowing and breathing atrophy and die.
There is still no cure for the fatal disease, which affects 30,000 Americans, according to the National Institutes of Health.
But research has yielded promising findings - last week UI scientists published their study of a drug that doubled the average life spans of mice with the inherited form of the disorder. The report was published online last week in the Journal of Clinical Investigation.
"It transfers to the quality of life," said lead scientist John Engelhardt, a UI professor and the head of the anatomy/cell-biology department. "There's potential that the drug could be helpful."
The scientists discovered a protein interaction that leads to overproduction of a molecule that is damaging in high quantities and linked to neurodegenerative diseases.
The protein complex works like a furnace with a broken thermostat that produces too much heat, Engelhardt said. A drug called apocynin hinders production of the culprit molecule by disabling one component.
In an earlier study, Engelhardt and his team worked directly with genes to double the life span of mice. Apocynin, which can be extracted from plants and easily made in labs, also achieved such effects when fed to the mice through their water.
"It's turning off the 'furnace' by a different method," he said.
The disease usually strikes between age 40 and 60, and it is more common in men than women. Approximately half of people diagnosed with the illness live more than three years, and only 10 percent more than 10 years.
After the Jan. 24 study was published, Engelhardt found himself poring over the disease's Therapy Development Institute's website, where patients read published research and comment on the blog. The study has already generated hundreds of posts.
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