neuromuscular junction NMJ NOGO-A ALS


Staying connected. GSK's ozanezumab may enable motor neuronal axons damaged by ALS to be repaired and reconnected to muscle fibers by mopping up Nogo-A (an axon growth blocker) in the surrounding debris. Image: JieFei Yang PhD, Salk Institute.

Motor neurons signal our muscles to move. But in people with ALS, the neuromuscular junctions that transmit these electrical messages crumble leading to muscle weakness, paralysis and respiratory failure.

Researchers are working hard to develop medicines that protect these structures in hopes to slow or stop the progression of disease.  One of these medicines, GlaxoSmithKline’s ozanezumab (GSK1223249), hopes to do just that by helping keep the motor nerves and muscle fibers connected.

Ozanezumab is currently being tested in the clinic. A 48 week phase II trial is ongoing.  Nearly 300 people with ALS are expected to participate worldwide.

ALS Today’s Michelle Pflumm PhD talked to participating neurologist Pierre-François Pradat MD PhD of the Hôpital de la Pitié-Salpétrière in Paris to learn more about ozanezumab and its potential to treat ALS going forward.


To find out about other emerging strategies to help keep muscles moving, check out CK-357, helping pALS live strong? and Exercise: stretching the limits of ALS care

Patient Resources

Study of Ozanezumab in the Treatment of Amyotrophic Lateral Sclerosis  Contact  ALS TDI  Website


Pradat, P.F. et al. (2011) Abnormalities of satellite cells function in amyotrophic lateral sclerosis. Amyotrophic Lateral Sclerosis 12(4), 264-271.  Abstract  |  Full Text  (Subscription Required)

Pradat, P.F. et al. (2007) Muscle Nogo-A expression is a prognostic marker in lower motor neuron syndromes. Annals of Neurology 62(1), 15-20.  Abstract  |  Full Text  (Subscription Required)

Jokic, N, Gonzalez de Aguilar, JL, Dimou, L, Lin, S, Fergani, A, Ruegg, MA, Schwab, ME, Dupuis, L and Loeffler, J.P. (2006) The neurite outgrowth inhibitor Nogo-A promotes denervation in an amyotrophic lateral sclerosis model.  EMBO Reports 7(11), 1162-1167.  Abstract  |  Full Text

Jokic, N. et al.(2005) Nogo expression in muscle correlates with amyotrophic lateral sclerosis severity. Annals of Neurology 57(4), 553-556. Abstract  |Full Text  (Subscription Required)

Further Reading

Krakora, D., Macrander, C. and Suzuki, M. (2012) Neuromuscular junction protection for the potential treatment of amyotrophic lateral sclerosis.  Neurology Research International 2012, 379657. Abstract  |  Full Text