Poster preview
ePoster
CEREBELLAR A1D-ADRENERGIC RECEPTORS MEDIATE STRESS-INDUCED DYSTONIA IN AN EA2 MOUSE MODEL
Pauline Bohne-Rybarskiand 6 co-authors
Ruhr University Bochum
FENS Forum 2026 (2026)
Barcelona, Spain
Board PS06-09PM-038
Presentation
Date TBA
Board: PS06-09PM-038
Event Information
Poster Board
PS06-09PM-038
Poster
View posterAbstract
Episodic ataxia type 2 (EA2) is a rare inherited neurological disorder, where patients suffer from permanent ataxia and severe episodes of motor dysfunction exhibited as dystonia. Despite other factors, physical and emotional stressors trigger these episodes reliably in both human and mouse models. We used the well-established EA2 mouse model tottering to explore the involvement of cerebellar adrenergic receptors (AR) in the formation of stress-induced dystonic attacks. We found that specifically α1-ARs, which are differentially expressed on cerebellar Purkinje cells (PCs), are reliably activated by norepinephrine (NE) and required for initiation of dystonia, while α2-ARs are not. Moreover, both the pharmacological blockade of α1D-ARs using thesubtype specific antagonist BMY-7378, as well as the shRNA-induced knock down of selectively cerebellar α1D-ARs, were sufficient to effectively prevent stress-induced dystonia in tottering mice, but had no impact in reducing ataxia. In vivo recordings and live calcium (Ca2+) imaging of PCs demonstrated that α1D-AR blockade successfully protects PCs from NE-mediated erratic firing patterns through decreased release of Ca2+ from intracellular stores, thus preventing stress-induced dystonia. Together, our data show the modulatory effects of NE on dystonia severity and suggest a predominant role of cerebellar α1D-ARs in the formation of stress-induced dystonia in tottering mice.
Recommended posters
NORMAL COGNITIVE FUNCTION AND SOCIAL BEHAVIOR IN HETEROZYGOUS TOTTERINGTG/- MICE
Mareike Josten, Klara Kleim, Melanie Mark
INCREASED CORTICOSTERONE LEVELS AND ENHANCED RESPONSE TO ACUTE STRESS IN A SCA3 MOUSE MODEL
Joana Correia, Daniela Monteiro-Fernandes, Sara Guerreiro, Bruna Ferreira-Lomba, Daniela Cunha-Garcia, Patrícia Gomes, Andreia Teixeira-Castro, Patrícia Maciel, Sara Duarte-Silva
ANATOMICAL AND FUNCTIONAL PROFILING OF SEROTONERGIC CIRCUITS IN MACHADO-JOSEPH DISEASE
Cármen Vieira, Daniela Monteiro-Fernandes, Sara Duarte-Silva, Jonas Campos, Nuno Dinis Alves, Ana João Rodrigues, Lin Tian, Kamran Khodakhah, Patrícia Maciel, Carina Soares-Cunha, Andreia Teixeira-Castro
EFFICACY OF CHRONIC 5-HT1A RECEPTOR AGONISM BY NLX-112 IN A MOUSE MODEL OF SPINOCEREBELLAR ATAXIA TYPE 3
Bruna Ferreira-Lomba, Sara Guerreiro, Sara Duarte-Silva, Daniela Cunha-Garcia, Stéphanie Oliveira, Cármen Vieira, Joana Pereira-Sousa, Daniela Vilasboas-Campos, André Vidinha-Mira, Daniela Monteiro-Fernandes, Jonas Campos, Mark A. Varney, Mark S. Kleven, Adrian Newman-Tancredi, Andreia Teixeira-Castro, Patrícia Maciel
CEREBELLAR CONTROL OF CORTICO STRIATAL PLASTICITY & THERAPEUTIC CEREBELLAR DEEP BRAIN STIMULATIONS IN DYSTONIA
Maria Patricia Anez, Romain Sala, Clément Léna, Daniela Popa
ELECTROPHYSIOLOGICAL CHARACTERIZATION OF DYSFUNCTION IN PURKINJE NEURONS IN A NEW MOUSE MODEL FOR CHILDHOOD-ONSET CEREBELLAR ATROPHY
Sudeepta Sarkar, Theo Petrizzelli, Sophie Nicole, Philippe Lory, Philippe Isope