Body’s own Defense Against ALS Actually Drives Disease Progression at Later Stages
ALS (Amyotrophic Lateral Sclerosis) is also known as Lou Gehrig’s disease, is a lethal disease which does not have any effective treatment till now. In ALS, first the motor neurons (guide the muscle movement) are attacked and slowly the disease spreads to other types of cells in the spinal cord. Over time, ALS patients experience inability to speak, move and face difficulty in breathing. The biggest barrier to treat ALS is its progression which is very dynamic in nature. In almost all instances patients within 5 years of diagnosis, succumb to ALS.
Researchers at Mortimer B. Zuckerman Mind Brain Behaviour Institute, Columbia discovered that the cellular cleanup process – autophagy (body’s natural defense) suppresses the progression of disease in the early stages but advances the deadly spread throughout the spinal cord in the later stages. The authors argue, when autophagy is intact, other cell types neighboring motor neurons undergo an inflammatory reaction and contribute to the faster progression of ALS but in the absence of autophagy the disease spread is slower. The study is published in the Proceedings of the National Academy of Sciences.
These studies provide a path into early stages of ALS and new insights to its complexity. Additionally, the study can help researchers to detect and treat ALS before the onset of destructive symptoms.