# Mitochondrial function and dysfunction in hair cells

> **NIH NIH R01** · UNIVERSITY OF WASHINGTON · 2020 · $322,284

## Abstract

PROJECT SUMMARY
Genetic disruptions of over thirty genes affecting mitochondrial functions result in hearing loss. We propose to
understand the cellular consequences of representative mutations in mechanosensory hair cells. Our
preliminary studies demonstrate that hair cells undergo rapid mitochondrial biogenesis as they mature, and
that mitochondria actively respond to mechanosensory cues. We propose studies to understand the regulation
of mitochondrial growth and function, focusing on the roles of specialized connections between mitochondria
and the endoplasmic reticulum (ER). Growing evidence supports a role for ER-mitochondrial junctions
regulating mitochondrial metabolism, dynamics, and stress and for playing a central role in regulated cell death.
We propose experiments using live imaging of hair cells in the zebrafish lateral line, a system amenable to
study of cellular functions in vivo. We will assess mitochondrial growth, oxidation and ATP utilization in hair
cells, and determine the consequences of disrupting ER-mitochondrial communication. We will measure
changes in mitochondria in response to mechanical stimuli. Using CRISPR, we will generate zebrafish
models of human mitochondrial mutations to assess functional consequences for hair cells. Our experiments
will reveal potentially fundamental mechanisms of mitochondria in hair cells and how these mechanisms go
awry in human hearing loss.

## Key facts

- **NIH application ID:** 9822159
- **Project number:** 5R01DC015783-04
- **Recipient organization:** UNIVERSITY OF WASHINGTON
- **Principal Investigator:** David W Raible
- **Activity code:** R01 (R01, R21, SBIR, etc.)
- **Funding institute:** NIH
- **Fiscal year:** 2020
- **Award amount:** $322,284
- **Award type:** 5
- **Project period:** 2016-12-01 → 2021-11-30

## Primary source

NIH RePORTER: https://reporter.nih.gov/project-details/9822159

## Citation

> US National Institutes of Health, RePORTER application 9822159, Mitochondrial function and dysfunction in hair cells (5R01DC015783-04). Retrieved via AI Analytics 2026-05-22 from https://api.ai-analytics.org/grant/nih/9822159. Licensed CC0.

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