720 results found
Misfolded SOD1 in ALS pathogenesis
Project Overview
The first genetic cause of ALS to be discovered (in 1993) encoded a mutation in protein called superoxide dismutase 1 (SOD1). Decades later, it is largely agreed upon that the abnormal shape (called misfolding) of SOD1 mutants is at the root of toxicity in some forms of ALS. Traditionally, misfolded…
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See the project and researchersStudy of the impact of glycation on ALS using an in vitro tissue-engineered model of spinal cord
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Tissue engineering is a process that was originally designed and continues to be used for growing cells outside the body, turning them into functional tissues and organs, and applying them for clinical use. Skin grafting and developing new organs for transplant are examples of how tissue engineering are used as…
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See the project and researchersRGNEF modulates protein misfolding in ALS
Project Overview
Discoveries made in Canada by the lab of Dr. Michael Strong over the past few years have implicated a protein called Rho guanidine nucleotide exchange factor (RGNEF) both pathologically and genetically in causing ALS. Further study demonstrated that RGNEF could play a role in the abnormal shape (misfolding) and toxicity…
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See the project and researchersMuscle-targeted therapy for ALS
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In ALS, the disease is characterized by degeneration of motor neurons and an inability of the brain to signal the muscles to move, resulting in paralysis. Over the past decade, it has become clear that motor neurons are not the only cell type involved in the disease process and attempting…
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See the project and researchersRegulation of endosomal membrane trafficking by C9ORF72 in ALS
Project Overview
Dr. Peter McPherson, professor at the Montreal Neurological Institute, McGill University will receive one year of support to work on the connection between the most prominent known cause of ALS, a mutation in a gene called C9ORF72, and a previously unrelated critical cellular function called endosomal membrane trafficking. Endosomal membrane…