Cell recycling system damaged in ALS

A breakdown of a recycling system in cells appears to be the underlying cause of ALS (most common form of motor neuron disease)


The breakdown occurs in the recycling system in the nerve cells of the spinal cord and the brain. In order to function properly, the protein building blocks in the cells need to be recycled. But in ALS, that system is broken. The cell cannot repair or maintain itself and becomes severely damaged.

The scientists found a protein, ubiquilin2, which should be directing the recycling process, does not work in people with ALS. This means the damaged proteins accumulate in nerve cells of the spinal cord and brain, causing their degeneration.


The researchers, from Northwestern University Feinberg School of Medicine, found this breakdown occurs in all three forms of ALS.


It's been known for some time that the waste and recycling system in motor neurons is damaged, but this is the first time that there has been direct proof. Now researchers can test for drugs that would regulate this protein pathway, so it functions as it should in a normal state. This discovery provides researchers with an exciting new avenue to explore as they search for an effective treatment.(22)