Protein buildup in ALS brains linked to lower protein removal factor

Researchers at Torrey Pines Institute for Molecular Studies, in Florida, discovered that patients with amyotrophic lateral sclerosis (ALS) have lower levels of a factor that controls autophagy, a cell mechanism for protein removal from the brain.

The study, “Transcription Factor EB Is Selectively Reduced in the Nuclear Fractions of Alzheimer’s and Amyotrophic Lateral Sclerosis Brains,” published in Neuroscience Journal, suggests that the lower levels found in brain areas crucial for movement might contribute to protein buildup, which is believed to contribute to toxicity and neurodegeneration in ALS patients.

As people age, the mechanics of getting rid of old and dysfunctional proteins becomes less efficient. In neurons, a crucial part of the system focuses on larger structures such as aggregated proteins. The second process, called autophagy, captures proteins in a membrane that engulfs the protein marked for destruction. The structure, called an autophagosome, then fuses with another cell component called lysosome, which contains enzymes that chop the protein to pieces.

Researchers are increasingly realizing that the system is either dysfunctional or insufficient in ALS, because of evidence that shows proteins aggregate in ALS patients just as they do in other neurodegenerative diseases such as Alzheimer’s. 

excerpt © 2016 ALS News Today