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Article

Inhibition of Hedgehog signaling does not mitigate polycystic kidney disease severity in a Pkd1 mutant mouse model.

Journal of cell science - 15 Oct 2025

Gombart Sean K, Houghtaling Scott, Ho Tzu-Hua, Beier David R

Abstract excerpt

Autosomal dominant polycystic kidney disease (ADPKD) is a monogenic disorder caused by mutations in PKD1 or PKD2, encoding polycystin-1 and polycystin-2, respectively. These polycystins form a cilia-localized complex that, when mutated, fails to inhibit an uncharacterized cilia-dependent cyst activation (CDCA) signal. This leads to progressive bilateral cyst growth and ultimately compromised renal function....

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Share to Polycystic Kidney, Autosomal Dominant
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Methodology Data sources Editorial policy Privacy About Contact Helena Bioinformatics

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Source

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Identifiers

PMID: 40554762
DOI: 10.1242/jcs.264133
PMCID: PMC12377710

Genes

PKD1
PKD2

Provenance

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

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