# Molecular Mechanisms of Clarin-1 and Clarin-2 function in mechanosensory hair cells

> **NIH NIH R01** · JOHNS HOPKINS UNIVERSITY · 2024 · $717,894

## Abstract

SUMMARY
The long-term goal of my laboratory is to elucidate the mechanisms that regulate the development and function
of hair bundles of cochlear hair cells, and how defect in hair bundles cause disease. We propose here to study
the function of two genes, clarin1 (CLRN1) and clarin2 (CLRN2) that have been linked to hearing loss. Mutations
in the murine Clrn1 and Clrn2 genes cause defects in hair bundle development and MET. We hypothesize that
CLRN1 and CLRN2 form protein complexes with proteins that have previously been linked to hair bundle
development and MET to affect the development and function of hair cells. To test our hypothesis, we will use
genetically modified combined with immunohistochemistry, electron microscopy as well as biochemical, cell
biological and electrophysiological methods to study CLRN1 and CLRN2 function in hair cells. Our preliminary
data show the feasibility of our approach. We have identified CLRN1 and CLRN2 binding partners that are
encoded by genes linked to deafness and our findings suggest that these bindings partners act in concert with
CLRN1 and CLRN2 to regulate hair cell development and function.

## Key facts

- **NIH application ID:** 10937379
- **Project number:** 1R01DC021941-01
- **Recipient organization:** JOHNS HOPKINS UNIVERSITY
- **Principal Investigator:** Ulrich Mueller
- **Activity code:** R01 (R01, R21, SBIR, etc.)
- **Funding institute:** NIH
- **Fiscal year:** 2024
- **Award amount:** $717,894
- **Award type:** 1
- **Project period:** 2024-08-07 → 2029-04-30

## Primary source

NIH RePORTER: https://reporter.nih.gov/project-details/10937379

## Citation

> US National Institutes of Health, RePORTER application 10937379, Molecular Mechanisms of Clarin-1 and Clarin-2 function in mechanosensory hair cells (1R01DC021941-01). Retrieved via AI Analytics 2026-06-13 from https://api.ai-analytics.org/grant/nih/10937379. Licensed CC0.

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