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Ciliary ARL13B inhibits developmental kidney cystogenesis in mouse
Robert E Van Sciver1, Alyssa B Long1, Harrison G Katz1,2
1Department of Human Genetics, Emory University School of Medicine, 615 Michael Street, Suite 301, Atlanta, GA 30322, USA.
Biorxiv : the Preprint Server for Biology
|February 17, 2023
Summary
ARL13B protein, found in cilia, prevents kidney cysts in mice. Its function within cilia inhibits cyst formation, independent of its GEF activity for ARL3.
Area of Science:
- Cell Biology
- Developmental Biology
- Genetics
Background:
- Cilia are crucial cellular organelles involved in development and disease.
- ARL13B is a ciliary protein implicated in kidney development.
- Loss of cilia or ARL13B function leads to kidney cyst formation.
Approach:
- Investigated ARL13B function in mouse kidney development using genetically engineered variants.
- Examined mice with cilia-excluded ARL13B (ARL13BV358A) to assess localization-dependent function.
- Analyzed mice with impaired ARL13B GEF activity (ARL13BR79Q) to determine the role of ARL3 interaction.
Key Points:
- ARL13B exclusion from cilia resulted in cystic kidneys, indicating its function is ciliary.
- ARL13B variants lacking ARL3 GEF activity did not cause kidney cysts, suggesting this function is dispensable.
- ARL13B acts within cilia to prevent renal cystogenesis.
Conclusions:
- ARL13B's role in inhibiting renal cystogenesis is localized to cilia.
- The GEF activity of ARL13B towards ARL3 is not required for its function in preventing kidney cysts.
- ARL13B is a critical regulator of kidney development, acting via a ciliary mechanism independent of its GEF activity.
