August 22, 2011

Cell Recycling Breakdown Possibly Responsible For Onset Of ALS

Researchers from the Northwestern University Feinberg School of Medicine may have pinpointed the cause of Amyotrophic lateral sclerosis (ALS), according to BBC News reports on Sunday.

ALS, which the BBC referred to as "the most common form of motor neuron disease," could be the result of "a breakdown of a recycling system in cells."

The Northwestern researchers "found the flaw in the way nerve cells in the brain recycle protein building blocks, which means cells cannot repair themselves and become damaged," according to the British news organization report.

"The breakdown occurs in the recycling system in the nerve cells of the spinal cord and the brain," the BBC News story reported. "In order to function properly, the protein building blocks in the cells need to be recycled“¦ But in ALS, that system is broken. The cell cannot repair or maintain itself and becomes severely damaged."

The key element is a protein known as ubiquilin2, which typically directs this "recycling" process, but does not function properly in those suffering from ALS. As a result, "the damaged proteins accumulate in nerve cells of the spinal cord and brain, causing their degeneration."

The results of the study have been published in the journal Nature.

According to the ALS Association, also known as "Lou Gehrig's Disease" in honor of the famous baseball player who suffered from the disorder, is "a progressive neurodegenerative disease that affects nerve cells in the brain and the spinal cord."

"Motor neurons reach from the brain to the spinal cord and from the spinal cord to the muscles throughout the body," the organization's website adds. "The progressive degeneration of the motor neurons in ALS eventually leads to their death. When the motor neurons die, the ability of the brain to initiate and control muscle movement is lost. With voluntary muscle action progressively affected, patients in the later stages of the disease may become totally paralyzed.

The ALS Association reports that more than 5,600 Americans are diagnosed with the disease each year, and the BBC adds that 350,000 people worldwide suffer from the disorder, which is often fatal within the first three years of its onset.

"This opens up a whole new field for finding an effective treatment for ALS," lead author Teepu Siddique told BBC News on Sunday. "We can now test for drugs that would regulate this protein pathway or optimize it, so it functions as it should in a normal state."

"This is a big news story for motor neuron disease research," added Dr. Belinda Cupid, chief of research and development at the UK's Motor Neuron Disease Association. "We've known for some time that the waste and recycling system in motor neurons is damaged, but this is the first time that there has been direct proof“¦ This discovery provides researchers with an exciting new avenue to explore as they search for an effective treatment."


On the Net: