ADELE | Early defects in the dynamic of the spinal sensorimotor network: is ALS a post-natal neurodevelopmental disorder ?

Summary
Amyotrophic lateral sclerosis (ALS) is a fatal and incurable neurodegenerative disorder caused by the loss of motoneurons in the brain and spinal cord. Each year, ALS is responsible for three-five deaths per 100000 people. ALS initiates in mid-life by muscle weakness, evolving rapidly into a generalized paralysis that leads irrevocably to death within 2-3 years of clinical onset. Despite an exploding knowledge of pathological processes acquired in adult subjects, successive clinical failures suggest that our way to consider and treat the pathology may not be the most pertinent and effective. The idea that the pathology can originate during development has received very little attention, while in the post-natal period the fundamentals of the sensorimotor system are being laid, its completeness will be central for the rest of our days. ADELE project proposes to explore ALS as a developmental disease that take roots during critical developmental stages when the synchronized activity of sensorimotor circuits is established, and that subclinical threshold defects remains silent before reaching a clinical break point. This conceptual progress, with diagnostic and early therapeutic implications, can be made possible with the recent access to network activity of the spinal cord in rodent neonates. ADELE proposes to 1) investigate the spatiotemporal network dynamics in ALS mouse neonates by recording field activity using multi-electrode array along the spinal laminae, 2) to complete this approach by behavioral study of proprioceptive integration, which is detrimental for spinal circuitry function and 3) identify activity modulating drug to restore early spinal network dynamics and subsequently influence the course of the disease. Our multidisciplinary strategy will fill knowledge gaps with the goal to propose new therapeutic intervention and open up new horizons for the patients.
Unfold all
/
Fold all
More information & hyperlinks
Web resources: https://cordis.europa.eu/project/id/882779
Start date: 01-04-2020
End date: 31-03-2022
Total budget - Public funding: 190 676,16 Euro - 190 676,00 Euro
Cordis data

Original description

Amyotrophic lateral sclerosis (ALS) is a fatal and incurable neurodegenerative disorder caused by the loss of motoneurons in the brain and spinal cord. Each year, ALS is responsible for three-five deaths per 100000 people. ALS initiates in mid-life by muscle weakness, evolving rapidly into a generalized paralysis that leads irrevocably to death within 2-3 years of clinical onset. Despite an exploding knowledge of pathological processes acquired in adult subjects, successive clinical failures suggest that our way to consider and treat the pathology may not be the most pertinent and effective. The idea that the pathology can originate during development has received very little attention, while in the post-natal period the fundamentals of the sensorimotor system are being laid, its completeness will be central for the rest of our days. ADELE project proposes to explore ALS as a developmental disease that take roots during critical developmental stages when the synchronized activity of sensorimotor circuits is established, and that subclinical threshold defects remains silent before reaching a clinical break point. This conceptual progress, with diagnostic and early therapeutic implications, can be made possible with the recent access to network activity of the spinal cord in rodent neonates. ADELE proposes to 1) investigate the spatiotemporal network dynamics in ALS mouse neonates by recording field activity using multi-electrode array along the spinal laminae, 2) to complete this approach by behavioral study of proprioceptive integration, which is detrimental for spinal circuitry function and 3) identify activity modulating drug to restore early spinal network dynamics and subsequently influence the course of the disease. Our multidisciplinary strategy will fill knowledge gaps with the goal to propose new therapeutic intervention and open up new horizons for the patients.

Status

CLOSED

Call topic

MSCA-IF-2019

Update Date

28-04-2024
Images
No images available.
Geographical location(s)
Structured mapping
Unfold all
/
Fold all
Horizon 2020
H2020-EU.1. EXCELLENT SCIENCE
H2020-EU.1.3. EXCELLENT SCIENCE - Marie Skłodowska-Curie Actions (MSCA)
H2020-EU.1.3.2. Nurturing excellence by means of cross-border and cross-sector mobility
H2020-MSCA-IF-2019
MSCA-IF-2019