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Application of Proteostasis Regulators in GABAA Receptor Misfolding Diseases

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Open Access
Article Type Mini Review
Subject Biology Group
OCLC JBRES Record
Xi Chen, Ting-Wei Mu* and Ya-Juan Wang*
Issue: Volume4-Issue11
Pages: 1640-1644
Received: 2023-11-13
Accepted: 2023-11-26
Published: 2023-11-28

Abstract

Protein homeostasis (proteostasis) relies on an orchestrated balance between protein synthesis, folding, assembly, trafficking, and degradation to ensure normal physiological function. Protein misfolding diseases often arise as a result of deficient proteostasis. Proteostasis regulators enhance the proteostasis network capacity to correct the folding, thus promoting the trafficking and restoring the function of disease-associated variants. Here, we focus on the application of proteostasis regulators to promote the folding, assembly, and trafficking of pathogenic γ-aminobutyric acid type A (GABAA) receptor variants. Since clinical variants in GABAA receptors often lead to neurological diseases, such as epilepsy, proteostasis regulators have the promise to be developed as novel therapeutics to target misfolding-prone GABAA receptors to treat their protein conformational diseases.

FullText HTML FullText PDF DOI: 10.37871/jbres1843 Crossmark

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© 2023 Xi Chen, et al. Distributed under Creative Commons CC-BY 4.0 Creative CommonsAttribution

How to cite this article

Chen X, Ting-Wei M, Ya-Juan W. Application of Proteostasis Regulators in GABAA Receptor Misfolding Diseases. J Biomed Res Environ Sci. 2023 Nov 28; 4(11): 1640-1644. doi: 10.37871/jbres1843, Article ID: JBRES1843, Available at: https://www. jelsciences.com/articles/jbres1843.pdf

Subject area(s)

Drug Metabolism

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