Study Explains Key Mechanism in ALS Development

Researchers have discovered a mechanism that leads to the degradation of upper motor neurons, cells linked to the development of amyotrophic lateral sclerosis (ALS), or Lou Gehrig’s disease.

In order to better understand the degredation of these neurons and its effect on development of ALS, researchers evaluated mice without the UCHL1 gene—a gene implicated in motor defects in humans.
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Researchers found that the loss of UCHL1 protein function increases stress in the endoplasmic reticulum, which causes degredation and the eventual death of upper motor neurons.

"Now that we appreciate the importance of upper motor neurons, we need to develop therapies that improve their survival. This study gives us a target to go after, bringing us one step closer to building effective treatment strategies," the researchers wrote.

"Survival requirements of these neurons cannot be ignored in ALS and in other diseases in which voluntary movement is impaired."

The complete study is published in the December issue of Cerebral Neurology.

-Michelle Canales

Reference:

Jara JH, Genc B, Cox GA, et al. Corticospinal motor neurons are susceptible to increased ER stress and display profound degeneration in the absence of UCHL1 function. Cereb Cortex. 2015 January [epub ahead of print] doi: 10.1093/cercor/bhu318.