Kidney-specific deletion of the BicC family RNA-binding protein 1 triggers an ADPKD-like cystogenic program

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Céline Gagnieux1, Florian Bernet1, Lona Kroese2

  • 1Ecole Polytechnique Fédérale de Lausanne (EPFL) SV ISREC, Station 19, 1015 Lausanne, Switzerland.

Iscience
|August 14, 2026
PubMed

Insights

The RNA-binding protein Bicc1 is crucial for preventing polycystic kidney disease (PKD). Loss of Bicc1 in specific kidney cells triggers aggressive cyst growth, highlighting its role in polycystic kidney disease pathogenesis.

Area of Science:

  • Nephrology
  • Molecular Biology
  • Genetics

Background:

  • Autosomal dominant polycystic kidney disease (ADPKD) is caused by mutations in PKD1 or PKD2.
  • Dysregulated cAMP and YAP signaling pathways contribute to cyst development in ADPKD.
  • BicC family RNA-binding protein 1 (Bicc1) deficiency in mouse embryos leads to cystic kidneys.

Purpose of the Study:

  • To investigate the role of Bicc1 in renal tubule development and polycystic kidney disease.
  • To establish conditional knockout models of Bicc1 in adult and developing kidneys.
  • To analyze the impact of Bicc1 deficiency on cyst formation and associated signaling pathways.

Main Methods:

  • Conditional knockout (cKO) of Bicc1 in renal tubules using Pax8-rtTA-Cre and Ksp-Cre systems.
  • Development and application of Automated Renal Tubule and Cyst Quantification (ARTCyQ) pipeline for image analysis.
  • Transcriptional profiling to identify molecular signatures in Bicc1-deficient kidneys.

Main Results:

  • Distal nephron-specific deletion of Bicc1 induced aggressive polycystic kidney disease-like phenotypes.
  • Bicc1 deficiency led to ambiguous nephron segment identity and activated ADPKD-related pathways, including YAP signaling.
  • Transcriptional profiling showed overlap with PKD and injury-repair signatures in Bicc1-deficient kidneys.

Conclusions:

  • Bicc1 plays a critical role in maintaining normal kidney development and preventing polycystic kidney disease.
  • Bicc1 deficiency activates injury-repair pathways and dysregulates signaling pathways implicated in ADPKD.
  • Bicc1 is integral to the polycystin signaling network in the kidney.
Keywords:
ADPKDAIARTCyQBicc1CREBDDX5YAPcystp68polycystic kidney disease

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