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Investigation of the innate immune system and motor neuron degeneration in genetic models of ALS

Principal Investigator:
  • Alex Parker,

    Centre hospitalier de l'Université de Montréal

  • ALS Society of Canada

Project Overview

In recent years, it was discovered that a microscopic worm called C. elegans could mimic some of the aspects of human ALS when they were engineered to have abnormal (mutant) genes that cause the disease. Not only do they have motor neuron degeneration like humans, but the worms experience paralysis. The ability of these worms to model ALS makes them a great tool to test drugs that might relieve these effects, but also to study the disease in a very efficient manner that might take more time and expense in more evolutionarily advanced animals like mice and rats. Through examination of these C. elegans ALS models, Dr. Parker has discovered that there is an abnormal increase in an immediate immune response that would typically be related to infection. Further work demonstrated that blocking this response actually reduced motor neuron degeneration in ALS model worms. In this study, Dr. Parker’s lab will attempt to understand how this immune response (called innate immunity) is activated by making mutant ALS genes and to further understand how it causes motor neurons to die. Identifying the pieces of this response that are crucial to disease in these worms will lead to further investigation in more advanced animal models of ALS and could provide new treatment targets to someday slow down the disease in humans.