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Article

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

Molecular therapy : the journal of the American Society of Gene Therapy - 2 Dec 2020

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

Abstract excerpt

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 models and patients' RPE cells limits our understanding of the pathological mechanism of RPE degeneration. In this...

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Share to Corneal Dystrophies, Hereditary
Share to Cytochrome P450 Family 4

Methodology Data sources Editorial policy Privacy About Contact Helena Bioinformatics

Share to Fatty Acids, Unsaturated

Share to Mice, SCID

Share to Pluripotent Stem Cells

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Source

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Identifiers

PMID: 32755565
DOI: 10.1016/j.ymthe.2020.07.024
PMCID: PMC7704739

Genes

RPE
CYP4V2

Provenance

PubMed metadata and Literature Corpus relationships. Corpus snapshot updated 5 Sept 2026.

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