Research Uncovers Cause of Lou Gehrig's Disease
Researchers at Northwestern University have uncovered the causes of the disease amyotrophic lateral sclerosis (ALS or Lou Gehrig's disease). ALS is a progressive neurological disease, characterized by gradual paralysis of muscles, and muscular wasting. ALS is often a fatal disease, as the respiratory muscles weaken, and the sufferers succumb to respiratory failure.
The most famous victim of this disease was Lou Gehrig, first baseman of the New York Yankees, who after 17 years playing for the Bronx Bombers, was forced to retire as a result of ALS. Two years after his retirement from baseball, Lou Gehrig died of ALS, at the age of 38. His name became synonymous with the disease.
ALS is a relatively common neurological disorder, especially among persons of European descent. A man's lifetime risk of developing this disorder is 1 in 350, and 1 in 420 for women. Up to the present day, this disease has been characterized by its difficult, progressive nature, and a relative lack of treatment options. Drug therapies focus mainly on symptom control, rather than slowing the progression of the disease.
Image courtesy of Chris Connelly
This latest research offers new hope that a treatment, and ultimately a cure for ALS may finally be in the offing. The root cause of this disease seems to be a deficiency in the system by which neurons recycle proteins in the spinal cord and the brain. The neuronal cells cannot repair or maintain themselves, and gradually these neurons become progressively more damaged. All three forms of ALS (hereditary, sporadic and ALS/dementia) seem to have the same deficiency in protein recycling in motor neurons. Knowing the root cause allows pharmaceutical researchers to develop targeted drug therapies to help regulate or optimize this protein pathway.