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Elucidating the basis for ubiquitination of misfolded ALS proteins by E3 ligase Enzymes

  • Program Type

    Team grants

  • Disease Area

    ALS

  • Competition

    ALS Canada - Brain Canada Discovery Grants

  • Province

    Ontario

  • Start Date

    2023

  • Total Grant Amount

    $125,000

  • Health Canada Contribution

    $62,500

Project Overview

A pathologic feature in both sporadic and familial ALS patients is the presence of large neuronal inclusions enriched with misfolded proteins, including SOD1, TDP43 and FUS that are frequently modified with the small protein ubiquitin (Ub). This observation, also common to Parkinson’s disease, indicates impairment in the ubiquitin-proteasome system that…

Neuromuscular proteins as potential biomarkers in ALS

  • Program Type

    Team grants

  • Disease Area

    ALS

  • Competition

    ALS Canada - Brain Canada Discovery Grants

  • Province

    Québec

  • Start Date

    2023

  • Total Grant Amount

    $300,000

  • Health Canada Contribution

    $150,000

Project Overview

Amyotrophic lateral sclerosis (ALS) is a neurodegenerative disease characterized by the loss of upper and lower motor neurons (MNs). Symptoms include muscle paralysis which rapidly progresses and ultimately results in respiratory failure within 2-5 years after diagnosis. Even if several genes causing ALS have been identified, still up to ~90%…

Axonal degeneration as a therapeutic target for ALS

  • Program Type

    Team grants

  • Disease Area

    ALS

  • Competition

    ALS Canada - Brain Canada Discovery Grants

  • Province

    Québec

  • Start Date

    2023

  • Total Grant Amount

    $300,000

  • Health Canada Contribution

    $150,000

Project Overview

One of the neuron’s most impressive morphological features is their long axonal projections, which are the basis of the complex, precise, and fast-firing neuronal network. To protect these fragile structures, neurons possess mechanisms to actively regulate axonal survival and degeneration, that are independent of the programmed cell death of the…

Modeling ALS progression by applying neuroinformatics to a novel in vitro human 3D tri-culture model of the disease

  • Program Type

    Team grants

  • Disease Area

    ALS

  • Competition

    ALS Canada - Brain Canada Discovery Grants

  • Province

    Québec

  • Start Date

    2023

  • Total Grant Amount

    $125,000

  • Health Canada Contribution

    $62,500

Project Overview

Amyotrophic Lateral Sclerosis (ALS) is a neurodegenerative disease in which the motor neurons degenerate, leading to symptoms of muscle weakness and progressive paralysis, resulting in the death of patients from respiratory failure. The development of disease modifying therapies have been largely unsuccessful due to the poor understanding into the mechanisms…

DNA damage driven motor disturbance in ALS: An ERVK integrase transgenic mouse model

  • Program Type

    Team grants

  • Disease Area

    ALS

  • Competition

    ALS Canada - Brain Canada Discovery Grants

  • Province

    Manitoba

  • Start Date

    2023

  • Total Grant Amount

    $124,900

  • Health Canada Contribution

    $62,450

Project Overview

Endogenous retrovirus-K (ERVK) is a genomic viral symbiont that contributes towards ALS neuropathology. Our collaboration proposes to examine how ERVK integrase (IN) enzyme activity drives DNA damage, TDP-43 deregulation and neuronal loss, leading to motor disturbances using a novel ERVK IN transgenic mouse model system. The intent of this proposal…