Accumulation of “Junk Proteins” Identified As One Cause of Aging and Possible Source of ALS

  • CNIO researchers provide a new hypothesis to understand the origin of amyotrophic lateral sclerosis, or ALS. It would be triggered by a similar problem to that occurring in a group of rare diseases called ribosomopathies.
  • In ALS patients, motor neurons would accumulate an excess of non-functional ribosomal proteins that eventually collapse the cell's clearance systems and cause toxicity.
  • The study also opens a new front in aging research. The authors provide experimental evidence that formally proves a kind of stress called “nucleolar stress” cause aging in mammals.

Amyotrophic lateral sclerosis (ALS) (Lou Gehrig’s disease) is a degenerative disease. The neurons responsible for movement begin to die and muscle control is progressively lost, leading to a fatal outcome. The causes of ALS are currently unknown, and there is no effective treatment. In a paper published March 22, 2024 in Molecular Cell, a team led by Óscar Fernández-Capetillo, PhD, Head of the Genomic Instability Group at the Spanish National Cancer Research Center (CNIO), provides the first evidence that a possible cause of the hereditary type of ALS –familial ALS– is the accumulation in motor neurons of “junk proteins,” proteins with no function that wrongly accumulate and prevent the cell from functioning properly. Specifically, these non-functional proteins that accumulate are ribosomal proteins, which normally form ribosomes, molecular factories in charge of protein production. The article is titled “Nucleolar Stress Caused by Arginine-Rich Peptides Triggers a Ribosomopathy and Accelerates Aging In Mice.”

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