Cell Death Pathways
Every cell in the body has a built-in suicide program that’s normally kept under control. Programmed cell death, known as apoptosis, can occur at various stages during development, or when a cell becomes too damaged by infection, cancer, or old age.
Studies by Packard scientists and others show that in ALS, however, this process occurs prematurely. In the SOD1 mouse model of the disease, scientists have verified that motor neurons ultimately die via apoptosis. More recent work by our researchers shows that the continued, early activation of apoptosis pathways occurs before ALS 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 by delaying 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 science and the therapeutic level.
