Figure 1.

In this image, some of the most relevant pathophysiological mechanisms in ALS are described. 1. Alterations in RNA and RNA-binding proteins such as TDP-43 or FUS, leading to aberrant cytoplasmic aggregates. 2. Increased free radicals that damage the motor neuron membrane, DNA, and mitochondria. 3. Alterations in protein degradation. 4. Defects in axonal transport. 5. Neuroinflammation of microglia that influences the progression of neurodegeneration (figure modified from [19]).