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Article

Valosin-containing protein in ciliary morphology: a novel target in ADPKD.

American journal of physiology. Renal physiology - 1 Aug 2025

Pioppini Carlotta, Bhardwaj Rishi, Schönauer Ria, Halbritter Jan, Hassan Fatima, Eckardt Kai-Uwe, Fedeles Sorin V, Yilmaz Duygu Elif, Krappitz Matteus

Abstract excerpt

Autosomal dominant polycystic kidney disease (ADPKD) is a hereditary disorder leading to kidney cyst formation and loss of kidney function. The major causative genes Pkd1 and Pkd2 encode for the ciliary proteins polycystin-1 (PC1) and polycystin-2 (PC2), respectively, which are involved in ciliary functions. Within PKD1-defective cells, the accumulation of misfolded PC1 proteins triggers the unfolded protein...

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Methodology Data sources Editorial policy Privacy About Contact Helena Bioinformatics

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Source

Identifiers

PMID: 40662578
DOI: 10.1152/ajprenal.00032.2025

Genes

VCP
PKD1

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PubMed metadata and Literature Corpus relationships. Corpus snapshot updated 7 Sept 2026.

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