$125,000 awarded to Dr. Jeehye Park, in collaboration with Dr. Hoon-Ki Sung, The Hospital for Sick Children.
Previous studies have indicated that increased metabolism (called hypermetabolism) is a common feature of ALS and can be associated with weight loss, which is often related to a poorer prognosis. The hypothalamus is the area of the brain responsible for regulating the body’s metabolism, and recent clinical studies suggest that this area may be compromised in people living with ALS.
Although these recent findings shed a light on a potential link between hypermetabolism and ALS, it remains unknown whether hypermetabolism actually contributes to the rate of disease progression. Moreover, little is known regarding how exactly the hypothalamus is damaged in ALS and whether this has a direct association with the disease.
With this grant, Dr. Park and her team aim to investigate these questions using a mouse model of ALS that carries mutations in the MATR3 gene. Dr. Park was previously awarded a 2016 ALS Canada-Brain Canada Career Transition Award and a 2019 ALS Canada Project Grant to develop and validate this mouse model and showed that these mice not only mimic the neurodegeneration seen in ALS, but also display signs of defects in the hypothalamus and an inability to gain weight.
By gaining a better understanding of the link between hypermetabolism and weight loss in ALS, and the biological pathways underlying hypothalamic dysfunction, these scientists should be able to pinpoint new targets and strategies for the treatment of ALS.