# Role of Cilia in Renal Fibrosis

> **NIH NIH R01** · YALE UNIVERSITY · 2020 · $376,875

## Abstract

Summary
The cilium is a widely distributed cell surface organelle that functions as a signaling hub for the vertebrate cell.
Ciliary defects leads to a wide range of human diseases collectively referred to as ciliopathies. Multiple
ciliopathies, including nephronophthisis, show renal fibrosis. However, the role of cilia in renal fibrosis has not
been studied extensively. In this project, we use Arl13b, a gene essential for cilia biogenesis, as an entry point
to dissect the role of cilia in renal fibrosis. We generated conditional Arl13b knockout mice. Our results show
that deletion of Arl13b in renal epithelial cells leads to renal fibrosis and cysts. Moreover, multiple signaling
pathways, including the Wnt, HH and Hippo pathway, are mis-regulated in the mutant kidney. This project
focuses on dissecting tissue specific functions of cilia and communications between different cell types in renal
fibrosis through both candidate and unbiased approaches. Aim 1 is focused on signaling in renal epithelial cells
regulated by ciliary defects and the relationship between different pathways. Aim 2 studies targets in interstitial
cells that are affected by ciliary defects and the functional significance of interstitial cilia. Through this study, we
will elucidate how ciliary defects in epithelial cells triggers a signaling cascade that eventually lead to renal
fibrosis and whether and how cilia in interstitial cells modify this response. These results not only will provide
insight to the mechanism of renal fibrosis in ciliopathies, but will also be informative to fibrosis in general.

## Key facts

- **NIH application ID:** 9937825
- **Project number:** 5R01DK113135-04
- **Recipient organization:** YALE UNIVERSITY
- **Principal Investigator:** ZHAOXIA SUN
- **Activity code:** R01 (R01, R21, SBIR, etc.)
- **Funding institute:** NIH
- **Fiscal year:** 2020
- **Award amount:** $376,875
- **Award type:** 5
- **Project period:** 2017-09-15 → 2022-05-31

## Primary source

NIH RePORTER: https://reporter.nih.gov/project-details/9937825

## Citation

> US National Institutes of Health, RePORTER application 9937825, Role of Cilia in Renal Fibrosis (5R01DK113135-04). Retrieved via AI Analytics 2026-05-23 from https://api.ai-analytics.org/grant/nih/9937825. Licensed CC0.

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