Amyotrophic lateral sclerosis (ALS) is a group of rare neurological diseases that mainly involve the neurons responsible for controlling voluntary muscle movement. The disease is perhaps progressive, and there is no cure or effective treatments. Early symptoms include muscle stiffness and the patients generally die of respiratory failure within 3 years.
When the researchers looked deep into the molecular physiology, they noticed a cellular change for causing ALS. As the nervous system damages, the star-shaped cell named astrocytes, behave strangely by undergoing a reactive change. Although some of this change is protective in nature, others become evil and damage the surrounding neurons. The reason for what causes the change in astrocytes still remains unknown.
To get a result, scientists used human induced pluripotent stem cells to compare with diseased individuals. Both normal and unusual astrocytes are compared to find out the root cause of change. Interestingly, they found that the reason for the change in the astrocytes was due to the removal of introns. These are non-coding sections of the genetic information. Altogether, the process is called splicing. In a healthy individual, the RNA pulls back introns and so there is not much splicing. Besides, when the introns are removed, the coding section sweeps around to develop proteins, which may result in the change of astrocytes.
A previous study has declared that splicing is reduced in ALS motor neurons, but in ALS astrocytes, it has increased resulting in only exons. This puzzling conclusion demanded upcoming clarification on it. This might be due to toxic immune insult to the immune cells when they got activated. Further research would make us clear and might help us in developing treatments for neurological disease conditions.
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Source: Medical Xpress
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