VANDEVELDELAB

We are focused on understanding the way by which neurons are lost in the fatal neurodegenerative disease, amyotrophic lateral sclerosis (ALS). ALS is also known as Lou Gehrig's Disease and Motor Neuron Disease... A portion of familial ALS results from mutations in superoxide dismutase 1 (SOD1) causing protein misfolding. The ALS research community largely agrees that misfolded SOD1 lies at the root of toxicity in familial ALS. We have demonstrated that several SOD1 mutants adopt a misfolded conformation that preferentially and progressively associates with the surface of spinal cord mitochondria prior to disease onset. We are further investigating the consequences of this association and exploring the concept that distinct forms of misfolded SOD1 exist.

Primary location: Montreal Canada