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New Candidate For ALS Cause

Less than three months after the Ice Bucket Challenge raised unprecedented amounts of money for research into amyotrophic lateral sclerosis, also known as Lou Gehrig’s disease, researchers have announced a breakthrough. Scientists are saying they have new insight into possible causes of the condition, and may have a clearer understanding than ever before of what is responsible for the degenerative disease. Previously, it was recognized that malfunctioning neurofilaments in the motor neurons did not properly connect these neurons to the muscles, so they muscles could not respond to the impulses meant to move them. but the precise reason for the malfunction was a mystery. Now it is believed that the malfunction arises proteins that are supposed to protect the neurons are unstable, often due to a genetic mutation.

Lou Gehrig’s disease affects an estimated 30,000 Americans. People with the condition first experience weakness in the extremities, and as the disease progresses—as the superoxide dismutase proteins start to weaken and break down—more and more muscles start to atrophy. It starts in the arms and legs where the motor neurons are largest, and eventually the muscles used for breathing are affected and suffocation ensues.

Different forms of the disease have longer or shorter prognoses, but ALS is currently a terminal condition with no known cure. However, with a new understanding of what causes the motor neurons to fail, it may be possible to develop better treatments.

Right now, patients with ALS are given palliative care to alleviate the pain and discomfort, and assistive technology is used to help them maintain independence. However, some stroke drugs are being investigated as ways to slow the progress of the disease and possible reverse some of the damage. Moreover, since the SOD protein is dependent on copper, the metal is being looked at as a potential treatment, with a possibility that it will help maintain the proteins. Copper is normally toxic, so ways must be found to administer it in a way that it is useful, without it poisoning the patient. Stem cell research using bone marrow is also being undertaken, with clinical trials showing some success in regrowing the motor neurons.

Potential Treatments For ALS

An estimated 30,000 Americans are affected by amyotrophic lateral sclerosis a neurodegenerative condition that causes the motor neuron cells in the spinal cord to gradually die off, keeping neural impulses from the brain from reaching the muscles. Muscle function slowly diminishes, making movement increasingly difficult. It is also known as Lou Gehrig's disease, after the New York Yankees player who was diagnosed with the condition in 1939, famously leading to his retirement from baseball, and who ultimately dies of the disease two years later. The condition typically begins with weakness in the arms and legs—the first symptom Gehrig noticed—and difficulty in breathing and swallowing. The muscles slowly begin to atrophy and eventually no longer work. In the end, the muscles needed for breathing no longer function.

The cause of ALS is unclear. Researchers believe it may be related to structures in the motor neurons called neurofilaments. In ALS, those neurofilaments are not constructed properly, so they malfunction. The task of the neurofilament is to transport molecules around motor neurons to enable the motor neurons to activate the muscles. The malfunctioning neurofilaments are unable to do this, so the molecules end up stuck, misplaced, or scattered. The researchers say the largest motor neurons, with the longest filaments, are in the legs, which is why the first signs of Lou Gehrig's disease are in the legs and feet.

There is no cure for ALS. Palliative care can make it possible for patients to live, and assistive technology allows them to prolong their independence, but nothing can be done to reverse or even stop the damage. However, there is some indication that an experimental drug for treating stroke patients may also disrupt a key mechanism of Lou Gehrig's disease. In experimental animals, this medication slowed down motor neuron death and delayed the progress of the disease.

Another possible treatment being investigated involves a copper compound that scientists hope will slow the condition and significantly improve the life expectancy of patients. Current treatments can extend lifespan by a handful of months at most. Since some of the damage ALS does has been traced to insufficient copper in the spinal cord, additional copper is thought to help. The compound allows the normally toxic metal to be administered safely; it is hoped this will prevent the damage from occurring, halting the disease.

Genes and Cells in ALS

Researchers in Texas may have found the mechanism behind the progressive neurodegenerative condition amyotrophic lateral sclerosis, also known as Lou Gehrig’s disease. The researchers say that a lack T cells, immune cells that help reduce inflammation, worsens the symptoms. Production of the cells is controlled by a gene called FoxP3; when the gene is inactive, the researchers found, the degeneration from the condition was more rapid.

ALS causes gradual diminishing of function as motor neuron cells in the spinal cord die off. The motor neurons are the medium through which the brain communicates with the muscles. The initial symptoms are weakness in the arms and legs, and difficulty in breathing and swallowing. Over time, the muscles atrophy and no longer work. There is not yet any known cure for the condition, which is estimated to affect about 15,500 Americans.

Medical research has established a link between inflammation and ALS, but that link is very complicated. Normally, inflammation is part of the process by which the body protects or heals from microbial invasion. It’s an important part of the healthy immune response. The death of motor neurons from ALS also seems to trigger inflammation, in the glial cells, but that inflammation seems in turn to worsen the effect on the motor neurons; the cells produce chemicals that damage the neurons surrounding them, damage that eventually destroys them.

While inflammation exacerbates disease in ALS patients, this inflammation is suppressed in some patients, said study author Jenny Henkel, a neurologist at Houston’s Methodist Neurological Institute, in a statement. “The data in our article suggest that regulatory T cells can suppress this inflammation.”

Another study, in Boston, found a type of cell in the blood that is associated with ALS. By looking for abnormal levels of these cells, called monocytes, doctors can detect ALS early and start treatment to slow its progression. Monoctyes also provide a line of investigation in the application of anti-inflammatory treatment to ALS.