Post-mortem studies of brain tissue from ALS patients reveal an abnormal form of tau is present in novel brain areas, and the tau interacts with DRP1. The tau appears to cause the brain cell's mitochondria to fragment and increase oxidative stress. Reducing tau reversed the effect, decreasing oxidative stress and mitochondrial fragmentation.
In patients with ALS, astrocytes within the brain become pro-inflammatory and tend to lose their protective function, resulting in changes in the ability to uptake glutamate.
Mislocalization of the TDP-43 protein alters the genetic instructions for UNC13A. The findings provide a potential new therapeutic target for the treatment of ALS and frontotemporal dementia.
Those working in production occupations, especially those exposed to volatile organic compounds, metals, combustion pollutants, and particulate matter have a higher risk of developing ALS.