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PSCs Reveal PUFA-Provoked Mitochondrial Stress as a Central Node Potentiating RPE Degeneration in Bietti’s Crystalline Dystrophy

Bietti’s crystalline dystrophy (BCD) is an incurable retinal disorder caused by the polypeptide 2 of cytochrome P450 family 4 subfamily V (CYP4V2) mutations. Patients with BCD present degeneration of retinal pigmented epithelial (RPE) cells and consequent blindness. The lack of appropriate disease m...

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Detalles Bibliográficos
Autores principales: Zhang, Zhao, Yan, Bin, Gao, Fei, Li, Qing, Meng, Xiaohong, Chen, Peikai, Zhou, Lei, Deng, Wen, Li, Cheng, Xu, Weiyi, Han, Shuo, Feng, Hong, Li, Yaping, Chen, Junhui, Yin, Zhengqin, Liao, Can, Tse, Hung-Fat, Xu, Aimin, Lian, Qizhou
Formato: Online Artículo Texto
Lenguaje:English
Publicado: American Society of Gene & Cell Therapy 2020
Materias:
Acceso en línea:https://www.ncbi.nlm.nih.gov/pmc/articles/PMC7704739/
https://www.ncbi.nlm.nih.gov/pubmed/32755565
http://dx.doi.org/10.1016/j.ymthe.2020.07.024