amyotrophic lateral sclerosis tagged posts

Scientists identify first compound to repair degenerating brain cells in paralyzing disease. Northwestern University scientists have identified the first compound that eliminates the ongoing degeneration of upper motor neurons that become diseased and are a key contributor to ALS (amyotrophic lateral sclerosis), a swift and fatal neurodegenerative disease that paralyzes its victims.

In addition to ALS, upper motor neuron degeneration also results in other motor neuron diseases, such as hereditary spastic paraplegia (HSP) and primary lateral sclerosis (PLS).

In ALS, movement-initiating nerve cells in the brain (upper motor neurons) and muscle-controlling nerve cells in the spinal cord (lower motor neurons) die. The disease results in rapidly progressing paralysis and death.

So...

A soft, gel-like substance called myelin envelopes the axons of neurons to shield them from damage. In ALS, the protective sheath is gradually stripped off, leaving axons bare, exposed and vulnerable to damage. Harvard Medical School researchers have identified a key instigator of this process.

Scientists have identified a key instigator of nerve cell damage in people with amyotrophic lateral sclerosis, or ALS. Researchers say the findings of their study may lead to new therapies to halt the progression of the uniformly fatal disease that affects more than 30,000 Americans. One such treatment is already under development for testing in humans after the current study showed it stopped nerve cell damage in mice with ALS.

The HMS study reveals that the aberrant behavior of an enzyme called RI...