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Article

CLRN1 Variants in Müller Cells Cause Mitochondrial Dysfunction in USH3A Retinal Organoids.

CNS neuroscience & therapeutics - 1 Aug 2026

Zhang Rui, Ji Xinbo, Yu Han, Xu Jingwen, Wang Yu, Sun Ping, Wang Yingxin, Tang Yao, Zhan Zexin, Jiao Yichang, Shan Didi, Lin Pengfei, Wang Dong-Dong, Zhao Yuying, Liu Xianyang, Yan Chuanzhu, Li Jianqiao, Li Mingfeng, Liu Fuchen, Hou Shengping

Abstract excerpt

BACKGROUND: Usher syndrome 3A (USH3A), caused by mutations in the CLRN1 gene, leads to retinitis pigmentosa and sensorineural hearing loss. While CLRN1's role in inner ear pathology is established, its contribution to retinal degeneration remains poorly understood. METHODS: Retinal organoids derived from a USH3A patient were analyzed using single-cell RNA sequencing and multi-electrode array recording. CLRN1...

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Methodology Data sources Editorial policy Privacy About Contact Helena Bioinformatics

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Identifiers

PMID: 42545893
DOI: 10.1002/cns.71068
PMCID: PMC13431285

Genes

CLRN1

Provenance

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

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