Scientists have discovered a new phenomenon in the brain that could explain the development of early stages of neurodegeneration that is seen in diseases such as ALS, which affects voluntary muscle movement such as walking and talking. The discovery was so novel, the scientists needed to coin a new term to describe it: mitoautophagy, a collection of self-destructive mitochondria in diseased upper motor neurons of the brain that begin to disintegrate from within at a very early age.

The discovery was so novel, the scientists needed to coin a new term to describe it: mitoautophagy, a collection of self-destructive mitochondria in diseased upper motor neurons of the brain that begin to disintegrate from within at a very early age. Upper motor neurons in the brain are responsible for initiating muscle movement and relaxation and are one of the first to break down in neurodegenerative diseases.

The study will be published on November 7 in the journal Frontiers in Cellular Neuroscience.

The phenomenon is observed mainly in one of the most common pathologies observed in neurodegenerative diseases, TDP-43 pathology, which is seen in more than 90% of ALS cases. When a pathology is present in the body, it indicates that something is wrong or functioning abnormally.

«I think we have found the culprit that primes neurons to become vulnerable to future degeneration: suicidal mitochondria,» said senior study author Hande Ozdinler, associate professor of neurology at Northwestern University Feinberg School of Medicine. «The mitochondria basically eat themselves up very early in the disease. This occurs selectively in the neurons that will soon degenerate in patient’s brains.»

«This type of degeneration begins much earlier than previously thought,» said study lead author Mukesh Gautam, the A Long Swim (ALS) Ellen Blakeman fellow at Northwestern.

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Materials provided by Northwestern University. Original written by Kristin Samuelson. Note: Content may be edited for style and length.