α-Crystallin chaperone mimetic drugs inhibit lens γ-crystallin aggregation: Potential role for cataract prevention

Islam, Sidra; Michael, T.; Frank, Brett S.; Hom, Grant L; Wheeler, Samuel; Fujioka, Hisashi; Wang, Benlian; Minocha, Geeta; Sell, David R.; Fan, Xingjun; Lampi, Kirsten J.; Monnier, Vincent M.

doi:10.1016/j.jbc.2022.102417

Cited by 6 publications

(2 citation statements)

References 84 publications

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“…These and other technological advances can support and lead to new therapeutic approaches e.g. (417,418) to protect image formation and improve the quality of life for the elderly. A lens is much more than an optical element in the visual system.…”

Section: Discussionmentioning

confidence: 99%

Insights into the biochemical and biophysical mechanisms mediating the longevity of the transparent optics of the eye lens

Quinlan

Clark

2022

Journal of Biological Chemistry

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Section: Discussionmentioning

confidence: 99%

Insights into the biochemical and biophysical mechanisms mediating the longevity of the transparent optics of the eye lens

Quinlan

Clark

2022

Journal of Biological Chemistry

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“…In the past several decades, researchers have been dedicated to achieve this goal. These include, for example, Islam et al screened small molecules that might protect gama-Crystallin from destabilization and aggregation after H 2 O 2 exposure, which provided insights for the existence of drugs and small molecules maintaining the transparency of lens [22]. Huang Y. et al found Biliverdin/Bilirubin redox pair could mitigate oxidative damage of LECs via NF-kB/iNOS and Nrf2/HO-1 pathways [23].…”

Section: Discussionmentioning

confidence: 99%

Identification of tRNA-derived small RNAs expression profiles and their potential functions in age-related cataract

Liu

Zhang

Jiang

et al. 2023

Preprint

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Object: To identify the expression profiles and demonstrate the potential functions of tRNA-derived small RNAs (tsRNAs) in the formation of age-related cataract (ARC). Methods: Human lens epithelial cell line (HLE-B3 cells) were treated with H2O2, small RNA sequencing analysis was performed to identify expression profiles of tsRNAs in control and H2O2-treated HLE-B3 cells. Then, GO and KEGG enrichment analysis were employed to illustrate the potential functions of differentially expressed (DE) tsRNAs and target genes in ARC. Finally, quantitative real-time PCR assay was used to verify the expression patterns of DE tsRNAs. Results: Totally 604 DE tsRNAs (329 upregulated and 275 downregulated) were obtained between control and H2O2-treated HLE-B3 cells. Bioinformatic analysis results showed that calcium signaling pathway may mainly participate in the formation of ARC. Conclusion: Our results indicated that tsRNAs may be associated with the pathogenesis of ARC, and thus might be potential diagnostic and therapeutic targets for ARC.

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The use of pharmacological chaperones in rare diseases caused by reduced protein stability

et al. 2022

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Rare diseases are most often caused by inherited genetic disorders that, after translation, will result in a protein with altered function. Decreased protein stability is the most frequent mechanism associated with a congenital pathogenic missense mutation and it implies the destabilization of the folded conformation in favour of unfolded or misfolded states. In the cellular context and when experimental data is available, a mutant protein with altered thermodynamic stability often also results in impaired homeostasis, with the deleterious accumulation of protein aggregates, metabolites and/or metabolic by‐products. In the last decades, a significant effort has enabled the characterization of rare diseases associated to protein stability defects and triggered the development of innovative therapeutic intervention lines, say, the use of pharmacological chaperones to correct the intracellular impaired homeostasis. Here, we review the current knowledge on rare diseases caused by reduced protein stability, paying special attention to the thermodynamic aspects of the protein destabilization, also focusing on some examples where pharmacological chaperones are being tested.

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α-Crystallin chaperone mimetic drugs inhibit lens γ-crystallin aggregation: Potential role for cataract prevention

Cited by 6 publications

References 84 publications

Insights into the biochemical and biophysical mechanisms mediating the longevity of the transparent optics of the eye lens

Insights into the biochemical and biophysical mechanisms mediating the longevity of the transparent optics of the eye lens

Identification of tRNA-derived small RNAs expression profiles and their potential functions in age-related cataract

The use of pharmacological chaperones in rare diseases caused by reduced protein stability

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