Neuromuscular junction on a chip tests ALS drugs

Researchers have created a human-derived neuromuscular junction from a person with amyotrophic lateral sclerosis (ALS) with the goal of testing anti-ALS drugs that not only prevent neuronal death but also improve muscle function (Sci. Adv. 2018, DOI: 10.1126/sciadv.aat5847). ALS is a neurodegenerative disease characterized by neuronal cell death, loss of muscle function, and paralysis, conditions that eventually lead to death. Two drugs have been approved for ALS treatment, but neither is optimally effective, says lead researcher Roger D. Kamm of Massachusetts Institute of Technology. Furthermore, current means of drug testing are two-dimensional and only give information about cell death. So Kamm and coworkers developed three-dimensional ALS-on-a-chip technology to better test muscle function. The device works by monitoring changes in the movements of microscopic pillars upon which muscle fibers grow. The team used it to test two drugs already in trials for treatment of ALS and found that both of them reduced cell death and improved muscle function. Kamm says that, in partnership with pharmaceutical maker Biogen, the team aims to create additional neuromuscular junctions from other people with ALS and test other compounds.