$100,000 awarded to Dr. Peter St. George-Hyslop at the University of Toronto.
Neurons are like long, living wires that connect to each other. They have an axon at one end and dendrites at the other. Electrical signals flow from the axon of one neuron to the dendrites of another neuron across gaps called synapses. For a healthy signal to cross the synapse, the right biological processes must occur in the axon and the dendrites. In ALS, motor neurons gradually break down and healthy signals no longer communicate with muscles.
In 2017, researchers discovered that mutations in a gene called annexin A11 (ANXA11) can be found in ALS. The protein expressed by this gene, called annexin A11, plays several roles in the body, including moving proteins to the right locations.
Recently, Dr. Peter St. George-Hyslop was a co-author of a study led by Dr. Michael Ward at the National Institute of Neurological Disorders and Stroke in the United States. They discovered that the annexin A11 protein acts like a seat belt to strap RNA and RNA-binding proteins to other cellular structures called lysosomes. This tethering allows the RNA and RNA-binding proteins to “hitchhike” to their final destinations in dendrites and axons, where they play an important role in maintaining their health. When annexin A11 protein is mutated, this tethering process malfunctions, impairing the normal movement of RNA and RNA binding proteins.
With this award, Dr. St. George-Hyslop will further explore how ANXA11 affects the proper biology of motor neurons. Findings from this project may provide new insights about the underlying mechanisms driving the development of ALS and potentially discover new treatment targets that help RNA and RNA binding proteins move to where they are needed.