Motor neurons activate unexpected defense to clear ALS-linked protein before it clumps
ALS is a disease in which, in most cases, a protein forms aggregates in neurons, leading to the rapid loss of motor control and death. A Nagoya University research team has discovered in human neuron cell cultures and mice an unexpected defense mechanism that neurons switch on to avoid these aggregates, offering a new potential path to developing a treatment.
Amyotrophic lateral sclerosis (ALS) is a neurodegenerative disorder characterized by a rapid loss of motor control and death, often due to the aggregation of a protein called TDP-43 in neurons. A Nagoya University research team has discovered that motor neurons can activate a protein-digesting machine called the immunoproteasome to prevent TDP-43 from clumping together and causing damage.
This newly found defense mechanism involves the release of interferon beta, which activates the immunoproteasome in neighboring motor neurons, breaking down the harmful protein before it can aggregate. The research, published in EMBO Reports, suggests that this pathway could potentially be targeted for the development of new treatments for ALS, although further studies are needed to determine its therapeutic potential.
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