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Amyotrophic Lateral Sclerosis from a cortical perspective: towards alternative therapeutic strategies

Project description

Amyotrophic lateral sclerosis: the role of upper motor neurons

Amyotrophic lateral sclerosis (ALS) is a rare and incurable neurodegenerative disease affecting the motor neurons that control voluntary muscle movements. ALS leads to the degeneration of both upper and lower motor neurons, resulting in severe prognosis and patient death within 2 to 5 years of diagnosis. Funded by the European Research Council, the CorticALS project aims to investigate the role of upper motor neurons in ALS. By exploring the dysfunction and loss of these neurons, the goal is to unravel molecular mechanisms that can be targeted in the context of new therapeutic strategies that protect or replace this specific neuronal type. This innovative approach could provide valuable insights and advance potential treatments for ALS patients.

Objective

Amyotrophic Lateral Sclerosis (ALS) is the most common adult-onset neurodegenerative disease of the motor system, with a prevalence of 2-3/100 000. In spite of intensive research efforts, ALS remains an incurable disease and presents with a very severe prognosis, leading to patient death within 2 to 5 years following diagnosis.
At the cellular level, ALS is characterized by the combined degeneration of both upper motor neurons (UMN, or corticospinal motor neurons) whose cell bodies are located in the cerebral cortex, and that extend axons to the medulla and spinal cord, and lower motor neurons (LMN, or spinal motor neurons) whose cell bodies are located in the medulla and spinal cord, and that connect to the skeletal muscles. This dual impairment allows to discriminate ALS from other, less severe diseases affecting either UMN or LMN. Despite this precise clinical description, it is striking to note that preclinical studies have so far mostly concentrated on LMN, leaving aside the role of UMN in ALS.
This project aims at shedding light on the contribution of the dysfunction and/or the loss of UMN in ALS, in order to design and test new therapeutic strategies based on the protection and/or the replacement of this exact neuronal type. This innovative question has never been directly asked so far. Our working hypothesis is that specific neurodegeneration of UMN, in the course of ALS, does not represent an isolated side effect, but rather actively contributes to the onset and progression of the disease. Based on the discovery of new molecular players, and the development of alternative therapies, this original thematic has the ambition to provide clinicians and patients with new answers and new therapeutic assets.

Host institution

INSTITUT NATIONAL DE LA SANTE ET DE LA RECHERCHE MEDICALE
Net EU contribution
€ 1 500 000,00
Address
RUE DE TOLBIAC 101
75654 Paris
France

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Region
Ile-de-France Ile-de-France Paris
Activity type
Research Organisations
Links
Total cost
€ 1 500 000,00

Beneficiaries (2)