Home > Amyotrophic Lateral Sclerosis > Destruction of motor neurons

Several cellular mechanisms are suspected of causing the destruction of motor neurons

  • Excitotoxicity: a mechanism of alteration and destruction of neurons by an excitatory substance called glutamic acid, present in the body. This mechanism is linked to a certain number of neurological diseases such as epilepsy, plaque sclerosis, Alzheimer’s, Parkinson’s … in ALS, etc. Riluzole acts on this mechanism by targeting excess glutamic acid. This is presently the only effective treatment.
  • Activation of immune cells which ensure that the defense and protection of the brain and spinal cord can bring about inflammation; it is responsible for the production of toxic substances for motor neurons
  • Mitochondria, the true batteries of cells, are especially used by motor neurons. Studies done on mice show that the earliest signs in the development of ALS are mitochondrial anomalies. These appear long before the muscular weakness.
  • Deregulation of mechanisms of apoptosis (programmed cell death). The presence of aggregates in the interior of cells is common in nearly all neurodegenerative diseases like Alzheimer’s, Parkinson’s and ALS. Preventing aggregate formation is integral to strategies of foreseeable treatments.
  • Cellular degeneration of oxidative stress management; when the balance of oxidants and antioxidants is deregulated and oxidants accumulate in the body, they can attack genes and cause mutations and modifications to the structure of DNA.
  • An interruption of protein transport along the axon, the elongation of the neuron which can reach several decimeters and paces the nervous influx. The motor neurons are very long cells since their axon can reach a length of up to a meter. The transport of proteins along that axon is therefore vital to the proper function of the cell. It guarantees the survival of the motor neuron and is considered in future treatments.

These different elements are the targets of preferred research and should allow for a greater understanding of the method of functioning of ALS and the development of an effective treatment.