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Open AccessArticle

Dynamics of a Protein Interaction Network Associated to the Aggregation of polyQ-Expanded Ataxin-1

1
Department of Biology, Aristotle University of Thessaloniki, 54124 Thessaloniki, Greece
2
Faculty of Biology, Johannes Gutenberg University, Biozentrum I, Hans-Dieter-Hüsch-Weg 15, 55128 Mainz, Germany
3
Institute of Applied Biosciences/Centre for Research and Technology Hellas, 57001 Thessaloniki, Greece
4
Department of Molecular Medicine and Surgery, Karolinska Institutet, 17177 Stockholm, Sweden
*
Author to whom correspondence should be addressed.
Genes 2020, 11(10), 1129; https://doi.org/10.3390/genes11101129
Received: 20 August 2020 / Revised: 14 September 2020 / Accepted: 23 September 2020 / Published: 25 September 2020
(This article belongs to the Section Technologies and Resources for Genetics)
Background: Several experimental models of polyglutamine (polyQ) diseases have been previously developed that are useful for studying disease progression in the primarily affected central nervous system. However, there is a missing link between cellular and animal models that would indicate the molecular defects occurring in neurons and are responsible for the disease phenotype in vivo. Methods: Here, we used a computational approach to identify dysregulated pathways shared by an in vitro and an in vivo model of ATXN1(Q82) protein aggregation, the mutant protein that causes the neurodegenerative polyQ disease spinocerebellar ataxia type-1 (SCA1). Results: A set of common dysregulated pathways were identified, which were utilized to construct cerebellum-specific protein-protein interaction (PPI) networks at various time-points of protein aggregation. Analysis of a SCA1 network indicated important nodes which regulate its function and might represent potential pharmacological targets. Furthermore, a set of drugs interacting with these nodes and predicted to enter the blood–brain barrier (BBB) was identified. Conclusions: Our study points to molecular mechanisms of SCA1 linked from both cellular and animal models and suggests drugs that could be tested to determine whether they affect the aggregation of pathogenic ATXN1 and SCA1 disease progression. View Full-Text
Keywords: ataxin-1; polyQ; pathway; network; drugs; blood-brain-barrier ataxin-1; polyQ; pathway; network; drugs; blood-brain-barrier
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MDPI and ACS Style

Vagiona, A.-C.; Andrade-Navarro, M.A.; Psomopoulos, F.; Petrakis, S. Dynamics of a Protein Interaction Network Associated to the Aggregation of polyQ-Expanded Ataxin-1. Genes 2020, 11, 1129. https://doi.org/10.3390/genes11101129

AMA Style

Vagiona A-C, Andrade-Navarro MA, Psomopoulos F, Petrakis S. Dynamics of a Protein Interaction Network Associated to the Aggregation of polyQ-Expanded Ataxin-1. Genes. 2020; 11(10):1129. https://doi.org/10.3390/genes11101129

Chicago/Turabian Style

Vagiona, Aimilia-Christina; Andrade-Navarro, Miguel A.; Psomopoulos, Fotis; Petrakis, Spyros. 2020. "Dynamics of a Protein Interaction Network Associated to the Aggregation of polyQ-Expanded Ataxin-1" Genes 11, no. 10: 1129. https://doi.org/10.3390/genes11101129

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