Start main content



Nov 26, 2018

Seminar - Neuroinflammation in a mouse model of amyotrophic lateral sclerosis with FUS gene mutation and effects of standard and new therapies (Speaker: Dr. Johannes de Munter)

Dr. Johannes de Munter, MD
Department of Neuroscience, School for Mental Health and Neuroscience
Maastricht University, Maastricht, Netherlands

Date: Monday, 26-November-2018
Time: 11:00 a.m.
Venue: Seminar Room 1, G/F, Laboratory Block, Faculty of Medicine Building
21 Sassoon Road, Pokfulam, Hong Kong

Amyotrophic lateral sclerosis (ALS) is a fatal motor neurodegenerative pathology that annually affects 1.9 per 100,000 of population worldwide. A novel transgenic mouse line, which is based on the mutation of Fused in sarcoma protein (FUS), DNA/RNA-binding factor, was proposed as a model of familial form of ALS (Shelkovnikova et al., 2013). Dr. de Munter’s team investigated behavioral, physiologic and molecular parameters of FUS-transgenic (FUS-tg) mice at their pre-symptomatic and symptomatic states. In addition, they studied the effects of chronic dosing with riluzole, a standard therapy of ALS, or selective COX2 blocker celecoxib, or a single i.c.v. administration of human stem cells. The latter treatment was previously shown to evoke anti-inflammatory effects in a rat model of spinal cord injury (de Munter, in preparation). Separate groups were acutely challenged with low dose of LPS. They found emotional and cognitive aberrations in FUS-tg mice at their pre-symptomatic stage, elevated plasma cytokines and increased behavioral and pro-inflammatory response to the LPS challenge. Pro-inflammatory changes were more pronounced in the brain than in the spinal cord. Neuro-Cells-treated animals improved signs of ALS pathology presumably cause of reduced neuroinflammation that suggests therapeutic potential of Neuro-Cells during ALS. A clinical trial is in progress for 2019.