学術雑誌論文

学術雑誌論文

text

iScience. 2024, 27(4), p. 109380

The deubiquitinase Otud7b suppresses cone photoreceptor degeneration in mouse models of retinal degenerative diseases

Elsevier Inc.

英語

text

2024-04-19

https://doi.org/10.1016/j.isci.2024.109380

Varner L.R., Chaya T., Maeda Y., et al. The deubiquitinase Otud7b suppresses cone photoreceptor degeneration in mouse models of retinal degenerative diseases. iScience 27, 109380 (2024); https://doi.org/10.1016/j.isci.2024.109380.

Primary and secondary cone photoreceptor death in retinal degenerative diseases, including age-related macular degeneration (AMD) and retinitis pigmentosa (RP), leads to severe visual impairment and blindness. Although the cone photoreceptor protection in retinal degenerative diseases is crucial for maintaining vision, the underlying molecular mechanisms are unclear. Here, we found that the deubiquitinase Otud7b/Cezanne is predominantly expressed in photoreceptor cells in the retina. We analyzed Otud7b−/− mice, which were subjected to light-induced damage, a dry AMD model, or were mated with an RP mouse model, and observed increased cone photoreceptor degeneration. Using RNA-sequencing and bioinformatics analysis followed by a luciferase reporter assay, we found that Otud7b downregulates NF-κB activity. Furthermore, inhibition of NF-κB attenuated cone photoreceptor degeneration in the light-exposed Otud7b−/− retina and stress-induced neuronal cell death resulting from Otud7b deficiency. Together, our findings suggest that Otud7b protects cone photoreceptors in retinal degenerative diseases by modulating NF-κB activity.

This article is licensed under a Creative Commons Attribution-NonCommercial-NoDerivatives 4.0 International License.

95642

The deubiquitinase Otud7b suppresses cone photoreceptor degeneration in mouse models of retinal degenerative diseases

Elsevier Inc.

英語

2024-04-19

iScience

27

4

109380

https://doi.org/10.1016/j.isci.2024.109380

https://hdl.handle.net/11094/95642