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The researchers identified unique cell types in the spinal cord of patients with amyotrophic lateral sclerosis (ALS) that can lead to early diagnosis.
Amyotrophic lateral sclerosis (ALS) is a neurodegenerative disease that affects neurons in the central nervous system. Also known as Lou Gehrig's disease, ALS is characterized by the progressive loss of motor neurons in the brain and spinal cord, resulting in paralysis, muscle degeneration and ultimately death. ALS presents a "focal appearance" whereby motor neuron degeneration begins in an area of the spinal cord and continues to the surrounding spinal regions. This usually corresponds to initial paralysis of the arms or legs that spreads to the rest of the body. Eventually, the disease can reach neurons that annihilate the lungs and disrupt the signals needed for breathing.
Current diagnostic options are daunting because of the lack of known biomarkers for ALS at an early stage. Researchers from the University of Illinois at Chicago have described the unique cellular changes that occur in the spinal cord during the progression of the disease. These changes may occur before the onset of symptoms and be an early sign of ALS. The authors used a new bioinformatic tool to badyze genomic spinal motoneuron data collected from patients who died from ALS. These samples were isolated in less affected areas of the spinal cord, representing early stage disease. Their results, published in the journal Neurobiology of the disease, clarifies the specific cell types present in ALS patients compared to healthy controls
"When we looked at the data, it was clear that the ALS patient cell mix was very different from that of patients with no neurodegenerative disease," said Dr. Fei Song, badociate professor of neurology and rehabilitation at the University of Toronto. the UIC College of Medicine author in the study.
Not only did ALS patients have different types of spinal motoneurons, but these diseased neurons were badociated with immune cells called microglia and macrophages. Evidence suggests that neuroinflammation, implicated in the pathogenesis of ALS, could be mediated by microglia and other immune cells. Although the exact mechanism by which inflammation causes neuron death is unclear, these findings support the hypothesis that motor neurons and immune cells interact during progression of the disease.
"Now that we have identified new subtypes of motor neurons and microglia present in ALS patients, we can begin to study in more detail their role in the progression of the disease," Song said. These motor neurons and / or disease-specific glial cells may be useful as novel biomarkers or therapeutic targets for slowing the progression of the disease.
Written by Cheryl Xia, HBMSc
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References:
- Dachet, F., Liu, J., Ravits, J. and Song, F. Predict motor neurons of the spine and glia diseases in sporadic ALS. Neurobiology of the disease 130104523 (2019).
- Parmet, S. The researchers describe new biomarkers of ALS, potential new drug targets. UIC today (2019).
Image by Arek Socha from Pixabay
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