Year: 2023
Type: article
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.
DOI: 10.37871/jbres1843
Authors: Xi Chen, Ting-Wei Mu*, Ya-Juan Wang*
Institution: Case Western Reserve University
Language: English
Topic: Heat shock proteins research
Subfield: Molecular Biology
Domain: Life Sciences
Open Access status: bronze
Funder: National Institutes of Health
Awards: G9000158106, G9000260302
OpenAlex ID: W4390957681