Amyotrophic lateral sclerosis, or ALS, is a progressive neurodegenerative disease that leads to
the death of nerve cells that control movement, leaving people unable to move and, eventually, to
breathe. ALS is fatal and, so far, incurable. Can new findings bring hope for novel therapies?
ALS, also known as Lou Gehrig disease, affects approximately 30,000 people in the United
Still, its causes remain largely unknown. There is currently no cure, and few treatments to
improve the quality of life or prolong life expectancy are available.
This condition is sometimes characterized by focal onset," meaning that symptoms appear one
by one in a particular area, such as an arm, before spreading to the rest of the body.
Thus, as motor neurons in the spinal cord and brain begin to die, a person with ALS May first
experience paralysis in one limb, then another, and so on until the motor cells that power the
muscles of respiration die, leaving the person unable to breathe.
Currently, No one test can provide a definitive diagnosis of ALS," according to the National
Institute of Neurological Disorders and Stroke and doctors eventually diagnose the condition
based on its symptoms.
For this reason, most people with ALS receive their diagnosis when the condition has already
progressed to a visible extent.
New research from the University of Illinois at Chicago has identified, for the first time, a set of
biomarkers that set people with ALS apart from those without a neurodegenerative disease.
These findings, which the investigators report in Neurobiology of Disease, could help doctors
diagnose the condition earlier, and they could also open up new avenues for research into
In the new study, the scientists reanalyzed samples of motor neurons and associated cells that
had been collected from the spinal cords of individuals who had died because of ALS and from
those of healthy people without a neurodegenerative condition.
The researchers had already looked at these samples in 2010 when they compared the cell
populations by analyzing gene expression in each. The researchers had collected the samples
from regions of the spinal cord that were less affected in people with ALS.
Since there must be cellular changes occurring in spinal cord regions adjacent to areas where
the disease has clearly affected motor neurons in the spine, we wanted to look at neurons from
these adjacent areas to determine if they are different from healthy tissue," explains lead
researcher Dr. Fei Song.
The debilitating disease has no effective treatment to stop the disease progression, and there are
only two medications that can prolong patient survival, by a few months. So, new drug targets,
especially ones that could be given in the earlier stages of the disease, are very much needed,"
The team had already identified some significant differences between the neurons and other cells
present in the spines of people with ALS and those present in the spines of healthy individuals.
In the current research, the scientists decided to reevaluate those samples using a novel method
of bioinformatics analysis to reassess the genetic data that they had initially gathered.
This allowed the team to identify specific types of cells within the collected samples. The
researchers thus found that people who had died from focal-onset ALS had different types of
motor neurons, compared with healthy individuals.
Moreover, these differences were associated with microglia and astrocytes, two types of
specialized neural cells that did not make an appearance in samples collected from the same
regions of the spinal cord in healthy participants.
When we examined the data, it was clear that the mixture of cells from the ALS patients was
very different from patients with no neurodegenerative disease," notes Dr. Song.
These findings, the team argues, could, in the future, allow them to better understand some of the
mechanisms underlying ALS and perhaps come up with targeted therapeutic strategies.
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