# The roles of Inturned in mammalian lung development

> **NIH NIH R03** · PENNSYLVANIA STATE UNIVERSITY, THE · 2022 · $80,250

## Abstract

Project Summary
 Congenital Lung Malformation (CLM) is a series of rare birth defects that in the most severe case are fatal at birth.
The effective prevention and early intervention of these defects require a comprehensive understanding of the mechanisms
underlying the morphogenesis of the embryonic lungs. The PI found that mouse embryos mutant for the CPLANE (cilia
and planar polarity effector) protein Intu exhibited severe defects in the growth and airway branching of the embryonic
lungs. The PI will test the hypothesis that loss of Intu leads to severe loss of cilia formation, hence compromised cilia-
dependent Hh and PDGF signaling. He will examine whether loss of Intu affects Hh and PDGF pathway outputs in vivo
and the response of the lung tissue to Shh and PDGF in culture. He will also test the genetic interaction between Intu and a
key regulator of Hh pathway, Gli2, in lung morphogenesis. Subsequently, He will examine the impact of Intu mutation on
signaling pathways and tissue patterning in embryonic lung development through a candidate gene approach, in which
known regulators of lung development will be examined. He will subsequently examine whether Intu mutation alters the
proliferation and programmed cell death in lung development. Finally, the PI will take a transcriptomics approach, to
compare the transcriptional profiles between wild type and Intu mutant lungs. The proposed research will reveal the roles
of CPLANE proteins and cilia in embryonic lung development, and contribute to the better understanding of lung diseases
such as CLM.

## Key facts

- **NIH application ID:** 10380773
- **Project number:** 5R03HD102462-02
- **Recipient organization:** PENNSYLVANIA STATE UNIVERSITY, THE
- **Principal Investigator:** AIMIN LIU
- **Activity code:** R03 (R01, R21, SBIR, etc.)
- **Funding institute:** NIH
- **Fiscal year:** 2022
- **Award amount:** $80,250
- **Award type:** 5
- **Project period:** 2021-04-05 → 2024-09-30

## Primary source

NIH RePORTER: https://reporter.nih.gov/project-details/10380773

## Citation

> US National Institutes of Health, RePORTER application 10380773, The roles of Inturned in mammalian lung development (5R03HD102462-02). Retrieved via AI Analytics 2026-05-23 from https://api.ai-analytics.org/grant/nih/10380773. Licensed CC0.

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