Discovered a mechanism that produces neuronal death in ALS patients
The amniotrofica lateral sclerosis (ALS ) is a neurodegenerative disease, Affecting neurons in the brain and spine , affecting control of voluntary movement in patients who suffer. It is estimated that approximately ten percent of cases the disease is hereditary.
In patients with hereditary disease has been established as a relationship between her and the mutation in a gene called SOD1 but so far nothing was known about mechanisms by which this gene triggered disease. Now , a new study published in the journal Neuron, is given to know the processes in which the gene is involved, and how they might be triggering neuronal death.
The study authors have found that, in pathological conditions, the SOD1 protein mutant is able to associate to an ion channel of mitochondria, inhibiting its activity. Mitochondria are responsible for the generation of energy required for the cell to survive. Having blocked the channel, the mitochondria would not work normally and producing reactive oxygen species, causing extensive damage to neurons in the spinal cord and leading eventually to death of them. These phenomena were observed in mice even before getting symptoms and were increased as they became more evident.
The results of this new research provide new clues to understanding how the neurodegenerative diseases and progression of the same over time, and to lay the groundwork for future research into therapies against them.
Found key molecule in neuronal death:
The molecule in question is called spermine and is involved in the type of neuronal death characteristic of neurodegenerative diseases like Alzheimer’s disease and acute brain damage such as epilepsy or ischemia.
Scientists Biomedical Research Institute Barcelona, Joint Center CSIC attached to the Institute of Biomedical Research August Pi i Sunyer have proved the involvement of spermine in excitotoxic neuronal death, an overexcitation of glutamatergic system of the brain that causes the death of neurons characteristic of acute and chronic brain damage. The study was published in the journal Journal of Neuroscience Research.
In addition, the molecule that belongs to the group of polyamines, is the subject of studies on the nervous system for its active role in glutamate receptors and is present in research on carcinogenic processes.
