Protein seen to have ‘housekeeping’ talents that protect neurons in diseases like ALS

A protein called Nrf2 could clear the harmful, misfolded proteins that cause Parkinson’s and other neurodegenerative diseases — including amyotrophic lateral sclerosis (ALS) — by activating “housekeeping” mechanisms within cells, according to a study in Parkinson’s models that was published in the Proceedings of the National Academy of Sciences.

Like other neurodegenerative diseases, Parkinson’s is caused by the accumulation of misfolded proteins in nerve cells that eventually kill the cells. In the case of Parkinson’s, these include the proteins alpha synuclein and LRRK2.

Using rat neurons and human neurons derived from induced pluripotent stem cells, a team of researchers led by Dr. Steven Finkbeiner, associate director and  senior investigator at Gladstone Institute of Neurological Disease in San Francisco, demonstrated that overexpressing Nrf2 in these nerve cells could accelerate the breakdown and clearance of mutant, misfolded alpha synuclein and LRRK2.

The researchers first expressed either mutant alpha synuclein or mutant LRRK2 in these neurons. They then reprogrammed the cells to overexpress Nrf2, and followed them for a week using an automated cell-tracking microscope that they had developed. This way, they were able to assess the proteins’ expression levels and overall health.

excerpt © 2017 ALS News Today. All rights reserved.