Project description DEENESFRITPL Investigating amyotrophic lateral sclerosis as a post-natal disorder Amyotrophic lateral sclerosis (ALS) is a fatal and incurable neurodegenerative disorder with a midlife onset. Muscle weakness rapidly develops into a generalised paralysis and leads to death within 2–3 years. Despite the substantial knowledge available on adult subjects, successive clinical failures suggest that current treatment methods may not be pertinent. The EU-funded ADELE project therefore intends to explore ALS as a developmental disease that takes root during a person’s critical developmental stages. To this end, it will investigate the spatiotemporal network dynamics in ALS mouse neonates and conduct a behavioural study of proprioceptive integration. It will further identify activity-modulating drugs to influence the course of the disease. The project will provide valuable insight into the development and treatment of ALS. Show the project objective Hide the project objective Objective 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. Fields of science natural sciencesbiological sciencesneurobiologysocial sciencespsychologybehavioural psychologymedical and health sciencesclinical medicineobstetricspostnatal caremedical and health sciencesbasic medicinepathologymedical and health sciencesbasic medicineneurologyamyotrophic lateral sclerosis Programme(s) H2020-EU.1.3. - EXCELLENT SCIENCE - Marie Skłodowska-Curie Actions Main Programme H2020-EU.1.3.2. - Nurturing excellence by means of cross-border and cross-sector mobility Topic(s) MSCA-IF-2019 - Individual Fellowships Call for proposal H2020-MSCA-IF-2019 See other projects for this call Funding Scheme MSCA-IF-GF - Global Fellowships Coordinator INSTITUT NATIONAL DE LA SANTE ET DE LA RECHERCHE MEDICALE Net EU contribution € 190 676,16 Address Rue de tolbiac 101 75654 Paris France See on map Region Ile-de-France Ile-de-France Paris Links Contact the organisation Opens in new window Website Opens in new window Participation in EU R&I programmes Opens in new window HORIZON collaboration network Opens in new window Other funding € 0,00 Partners (1) Sort alphabetically Sort by Net EU contribution Expand all Collapse all Partner Partner organisations contribute to the implementation of the action, but do not sign the Grant Agreement. FEDERAL STATE AUTONOMOUS EDUCATIONAL INSTITUTION OF HIGHER LEARNING KAZAN VOLGA REGION FEDERAL UNIVERSITY Russia Net EU contribution € 0,00 Address Kremlyovskaya 18 420008 Kazan See on map Activity type Higher or Secondary Education Establishments Links Contact the organisation Opens in new window Participation in EU R&I programmes Opens in new window HORIZON collaboration network Opens in new window Other funding € 92 322,24
