Causes of ALS: Switching on Cell Death Pathways

Every cell in the body has a built-in suicide program that’s normally kept under control. During embryonic development, and less commonly up into adulthood, the body uses this suicidal cell death pathway to eliminate cells that were temporarily needed. Before birth, for example, cell death – also called apoptosis – helps shape the embryo’s final form, much like taking down the scaffolding for a building. 

Studies by Packard scientists and others, however, show that in ALS, this process occurs prematurely. It’s been verified as the way that motor neurons ultimately die, for example, in the SOD1 mouse model of the disease.

More recent work by our researchers shows that chronic, early activation of apoptosis pathways occurs before symptoms appear in the model mice. The end stages of the process are turned on shortly before motor neuron death.

Drugs that inhibit apoptosis have shown some success in ALS mice – they delay disease onset and progress. These agents are not yet available for human use, although many companies are beginning to develop them. Understanding cell death pathways is an active area for future ALS research at both the basic and the therapeutic level.

Learn more about the research projects funded and coordinated by the Packard Center for ALS Research at Johns Hopkins, and targeted at finding the causes of ALS and a cure. Subscribe to ALS Alert to stay informed about ALS research and clinical trials.