THE ESSENTIAL
- Amyotrophic lateral sclerosis, also called Charcot disease, is a degenerative pathology resulting in paralysis of the voluntary motor muscles.
- A protein could be linked to the development of the disease.
- The protein, PEG10, would prevent communication between brain and nerve cells.
Amyotrophic lateral sclerosis, or Charcot disease, is a severe degenerative pathology, characterized by progressive paralysis of the muscles involved in voluntary motor skills. There is currently no cure, however certain medications moderately slow its progression.
An ancient protein linked to Charcot disease
Researchers at the University of Colorado-Bouder (United States) have discovered that a protein could play a role in the progression of amyotrophic lateral sclerosis. “Our work suggests that when this strange protein, known as PEG10, is present at high levels in nerve tissue, it changes the behavior of cells in ways that contribute to Charcot disease.”said Alexandra Whiteley, lead author of the study and assistant professor in the department of biochemistry at the University of Colorado-Bouder. The work was published in the journal eLife.
According to previous research, the PEG10 protein is involved in the development of the placenta in mammals. However, it can fuel certain pathologies such as cancers or Angelman syndrome, a neurological disorder, if it is present in excess in certain areas of the body.
PEG10 accumulation may be a hallmark of amyotrophic lateral sclerosis
During their study, the American research team discovered the proteins that accumulate during a mutation in the UBQLN2 gene, involved in the progression of amyotrophic lateral sclerosis. Among thousands of possible proteins, PEG10 tops the list.
Ensuite, scientists took spinal tissue from patients who died of Charcot disease. They then analyzed these proteins to determine which ones appeared to be overexpressed. Among more than 7,000 possible proteins, PEG10 ranked in the top five.
Thus, American researchers have highlighted that large quantities of PEG10 are present in the spinal cord tissues of patients affected by Charcot disease, which blocks communication between the brain and nerve cells. “It appears that PEG10 accumulation is a hallmark of amyotrophic lateral sclerosis”insisted Alexandra Whiteley. Before concluding: “The fact that PEG10 likely contributes to this disease means we may have a new target to treat Charcot disease.”
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