Amyotrophic Lateral Sclerosis Motor neuron diseases are
debilitating disorders that cause the loss of nerve cells in the
brainstem and spinal cord that control movement. Amyotrophic
lateral sclerosis (ALS), also known as Lou Gehrig’s disease,
accounts for 80 percent of motor neuron disease and occurs in
3-5 people per 100,000. This disease usually strikes people in
midlife and is relentlessly progressive, typically resulting in
death within five years of the first sign of symptoms. Patients
lose the ability to control their limbs, facial muscles,
swallowing, and eventually, the ability to breathe. While the
causes of ALS are largely unknown, most cases likely arise
through a combination of genetic factors and environmental
stress. A small number of ALS cases are due to known genetic
mutations that are transmitted through familial gene inheritance
(familial, or fALS). The genetic mutations known to cause ALS
have been transferred to cells and animal models so that the
disease may be studied more extensively in