Article

A <i>Drosophila</i> model for Dent’s disease reveals impaired ER export of Cubilin as pathogenic mechanism

2025-03-06

Abstract excerpt

Mutations in the CLCN5 gene encoding the chloride-hydrogen exchanger ClC-5 cause Dent’s disease, a genetic disorder of the endolysosomal pathway in the proximal tubules of the kidneys. Many patients also develop glomerular lesions, but the underlying mechanism is unclear. We have established an in vivo model for Dent’s disease using Drosophila nephrocytes that share similarities with podocytes and proximal tubu...

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Literature Corpus work: 89133d8d-6ddc-5ef1-8ca1-82a6640bd758
DOI: 10.1101/2025.03.05.641445

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