scholarly journals Limited ATF4 Expression in Degenerating Retinas with Ongoing ER Stress Promotes Photoreceptor Survival in a Mouse Model of Autosomal Dominant Retinitis Pigmentosa

PLoS ONE ◽  
2016 ◽  
Vol 11 (5) ◽  
pp. e0154779 ◽  
Author(s):  
Yogesh Bhootada ◽  
Pravallika Kotla ◽  
Sergei Zolotukhin ◽  
Oleg Gorbatyuk ◽  
Zsuzsanna Bebok ◽  
...  
Keyword(s):  
Mouse Model ◽  
Retinitis Pigmentosa ◽  
Er Stress ◽  
Autosomal Dominant ◽  
Autosomal Dominant Retinitis Pigmentosa

scholarly journals The Function of Drosophila USP14 in Endoplasmic Reticulum Stress and Retinal Degeneration in a Model for Autosomal Dominant Retinitis Pigmentosa

Biology ◽  
2020 ◽  
Vol 9 (10) ◽  
pp. 332
Author(s):  
Jung-Eun Park ◽  
Thị Xuân Thùy Trần ◽  
Nayoung Park ◽  
Jeonghun Yeom ◽  
Kyunggon Kim ◽  
...  
Keyword(s):  
Endoplasmic Reticulum ◽  
Retinitis Pigmentosa ◽  
Er Stress ◽  
Retinal Degeneration ◽  
Cellular Response ◽  
Autosomal Dominant ◽  
Metabolic Diseases ◽  
Autosomal Dominant Retinitis Pigmentosa ◽  
Alpha 1 Antitrypsin ◽  
The Unfolded Protein Response

Endoplasmic reticulum (ER) stress and its adaptive cellular response, the unfolded protein response (UPR), are involved in various diseases including neurodegenerative diseases, metabolic diseases, and even cancers. Here, we analyzed the novel function of ubiquitin-specific peptidase 14 (USP14) in ER stress. The overexpression of Drosophila USP14 protected the cells from ER stress without affecting the proteasomal activity. Null Hong Kong (NHK) and alpha-1-antitrypsin Z (ATZ) are ER-associated degradation substrates. The degradation of NHK, but not of ATZ, was delayed by USP14. USP14 restored the levels of rhodopsin-1 protein in a Drosophila model for autosomal dominant retinitis pigmentosa and suppressed the retinal degeneration in this model. In addition, we observed that proteasome complex is dynamically reorganized in response to ER stress in human 293T cells. These findings suggest that USP14 may be a therapeutic strategy in diseases associated with ER stress.

scholarly journals AAV Delivery of Wild-Type Rhodopsin Preserves Retinal Function in a Mouse Model of Autosomal Dominant Retinitis Pigmentosa

2011 ◽  
Vol 22 (5) ◽  
pp. 567-575 ◽  
Author(s):  
Haoyu Mao ◽  
Thomas James ◽  
Alison Schwein ◽  
Arseniy E. Shabashvili ◽  
William W. Hauswirth ◽  
...  
Keyword(s):  
Mouse Model ◽  
Retinitis Pigmentosa ◽  
Autosomal Dominant ◽  
Retinal Function ◽  
Wild Type ◽  
Autosomal Dominant Retinitis Pigmentosa

scholarly journals Erythropoietin Slows Photoreceptor Cell Death in a Mouse Model of Autosomal Dominant Retinitis Pigmentosa

PLoS ONE ◽  
2016 ◽  
Vol 11 (6) ◽  
pp. e0157411 ◽  
Author(s):  
Tonia S. Rex ◽  
Lorraine Kasmala ◽  
Wesley S. Bond ◽  
Ana M. de Lucas Cerrillo ◽  
Kristi Wynn ◽  
...  
Keyword(s):  
Cell Death ◽  
Mouse Model ◽  
Retinitis Pigmentosa ◽  
Photoreceptor Cell ◽  
Autosomal Dominant ◽  
Autosomal Dominant Retinitis Pigmentosa

Transgenic mice with a rhodopsin mutation (Pro23His): A mouse model of autosomal dominant retinitis pigmentosa

Neuron ◽  
1992 ◽  
Vol 9 (5) ◽  
pp. 815-830 ◽  
Author(s):  
Jane E. Olsson ◽  
Jon W. Gordon ◽  
Basil S. Pawlyk ◽  
Dorothy Roof ◽  
Annmarie Hayes ◽  
...  
Keyword(s):  
Mouse Model ◽  
Transgenic Mice ◽  
Retinitis Pigmentosa ◽  
Autosomal Dominant ◽  
Autosomal Dominant Retinitis Pigmentosa
Sign in / Sign up

Export Citation Format

Share Document