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DEEP CEREBELLAR NUCLEI CIRCUITRY IN AMYOTROPHIQUE LATERAL SCLEROSIS (ALS)
Aline Haettyand 10 co-authors
Inserm U1298
FENS Forum 2026 (2026)
Barcelona, Spain
Board PS03-08AM-030
Presentation
Date TBA
Board: PS03-08AM-030
Event Information
Poster Board
PS03-08AM-030
Poster
View posterAbstract
Amyotrophic Lateral Sclerosis (ALS) is a fatal neurodegenerative disease characterized by progressive motoneuron degeneration and paralysis. Increasing evidence suggests that circuit dysfunction beyond the motor cortex and spinal cord contributes to disease progression. Notably, early degeneration of inhibitory V1 interneurons, which directly innervate motoneurons, occurs before motoneurons death, suggesting a non-cell-autonomous component of neurodegeneration. Recent studies have identified direct projections from the deep cerebellar nuclei (DCN) to spinal V1 interneurons [1], yet their role in ALS remains unexplored. Using the ALS SOD1G93A mouse model, our data reveal early synaptic dysregulation in DCN neurons before ALS symptom onset, as early as P21. Furthermore, these synaptic alterations coincide with a significant decline in fine motor skills, as assessed by the single-pellet reaching task at P40. We hypothesize that early cerebellar circuit dysfunction initiates ALS pathophysiology. Our aim is to define the cellular and molecular mechanisms that disrupt DCN inhibitory synapse formation and organization. In addition, we will investigate how these alterations impact DCN to V1 interneuron connectivity, potentially driving V1 interneuron loss and early motor deficits. Understanding these mechanisms may provide new insight into cerebellar contribution to ALS and identify novel targets for therapeutic intervention.
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