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ARL13B-Cerulean rescues Arl13b-null mouse from embryonic lethality and reveals a role for ARL13B in spermatogenesis.

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Primary cilia shape postnatal astrocyte development through Sonic Hedgehog signaling.

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Related Experiment Video

Updated: Jan 9, 2026

Implementing Patch Clamp and Live Fluorescence Microscopy to Monitor Functional Properties of Freshly Isolated PKD Epithelium
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Ciliary ARLs drive renal cystogenesis.

Robert E Van Sciver1,2, Avery Forster3, Tamara Caspary1

  • 1Department of Human Genetics, Emory University School of Medicine, 615 Michael Street, Suite 301, Atlanta, GA 30322, USA.

Biorxiv : the Preprint Server for Biology
|December 3, 2025
PubMed
Summary

ARL13B

Keywords:
ARL GTPasesciliapolycystic kidney diseasepolycystins

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Area of Science:

  • Nephrology
  • Genetics
  • Cell Biology

Background:

  • Polycystic kidney disease (PKD) is a leading genetic cause of kidney failure.
  • Mutations in PKD1 or PKD2 cause PKD by affecting ciliary polycystin proteins.
  • The molecular drivers of the cilia-dependent cyst activation (CDCA) pathway remain unknown.

Purpose of the Study:

  • To investigate if ARL13B is a component of the CDCA pathway.
  • To determine the role of ARL13B's guanine nucleotide exchange factor (GEF) activity in PKD.
  • To elucidate the mechanism driving PKD pathogenesis.

Main Methods:

  • Utilized two distinct Arl13b mouse alleles (V358A and R79Q) in a Pkd1-deficient mouse model.
  • Assessed renal morphology, physiology (blood urea nitrogen), fibrosis, injury, and Wnt signaling.
  • Examined the impact of ciliary ARL13B localization and GEF activity on cyst development.

Main Results:

  • Loss of ciliary ARL13B or impaired GEF activity suppressed Pkd1-dependent kidney cysts.
  • Observed reduced kidney size, cystic index, and blood urea nitrogen levels.
  • Demonstrated suppression of renal fibrosis, injury, and Wnt signaling components (β-catenin, cyclin D1).

Conclusions:

  • ARL13B's GEF activity in cilia is a key mechanism driving the CDCA pathway in PKD.
  • Identified a specific subcellular location and molecular mechanism underlying Pkd1-dependent renal cystogenesis.
  • This study provides critical insights into the pathogenesis of polycystic kidney disease.