Until this week, most medical text books and online publications agreed that in 90- 95% of amyotrophic lateral sclerosis (ALS) cases, the disease occurs at random with no clearly associated risk factors.
Now, according to a study published in the Journal of Experimental Medicine, scientists have discovered two proteins that can conspire to promote the invariably fatal neurological disease.
ALS, or Lou Gehrig’s disease, is a rapidly progressive, devastating neurodegenerative disorder that results in progressive loss of motor function and ultimately death.
Jean-Pierre Julien and colleagues at Laval University in Quebec now find that a protein called TDP-43 binds to an inflammatory protein called NF-kB p65 in the spinal cords of ALS patients but not of healthy individuals.
TDP-43 and p65 were also more abundant in ALS than healthy spinal cords. It appears that TDP-43 and p65 cooperate to ramp up production of factors capable of promoting inflammation and killing nearby neurons.
Treatment of TDP-43 mice with Withaferin A, an inhibitor of NF-κB activity, reduced neuron loss and denervation and ALS disease symptoms.
These findings highlight p65 as a potential therapeutic target for this debilitating disorder which currently affects as many as 20,000-30,000 people in the United States and the additional 5,000 people who will be diagnosed with the disease each year. ALS is one of the most common neuromuscular diseases affecting people of all races and ethnic backgrounds. ALS most commonly strikes between 40 and 60 years of age, and men are affected more often than women.
SRxA’s Word on Health will be following this story and bringing you news on further advances in the fight against ALS, as they break.
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