# Copper Transport by Members of the Mitochondrial Carrier Family

> **NIH NIH R01** · AUBURN UNIVERSITY AT AUBURN · 2020 · $259,481

## Abstract

Copper is a key element in the survival and normal development of humans but it is also toxic to cells
when mishandled. In fact even mild disruption of copper homeostasis causes a wide array of disorders
including diabetes, cardiovascular disease, Alzheimer's disease, amyotrophic lateral sclerosis and Parkinson's
disease. The requirement for copper is in part due to its role as a cofactor in the terminal electron transport
chain complex cytochrome c oxidase. Though copper has an essential role in mitochondrial function, our
knowledge of how it is recruited to, and distributed within, the organelle is very limited. The hypothesis to be
tested here is that mitochondrial carrier family proteins that are used for translocation of substrates across the
inner membrane are critical to copper homeostasis. The proposed aims and experiments combine in vivo and
in vitro analysis of copper dependent phenotypes and transport assays to establish a role for a mitochondrial
carrier family protein in copper transport. We will demonstrate the copper transport function of this protein in
cell culture and an animal model by assessing the abundance and activity of copper-dependent enzymes, total
copper in mitochondria and total copper in whole cells. Data generated from the experiments proposed in
these aims will result in significant advances in our understanding of mitochondrial copper homeostasis in
humans. The impact of this proposal is that knowledge gained by identifying a mitochondrial copper transporter
can contribute to our understanding basic biology but also to a wide spectrum of diseases caused by copper
mishandling or mitochondrial dysfunction. Many of these diseases are currently untreatable and this proposal
will provide a potential druggable target for therapeutic intervention.

## Key facts

- **NIH application ID:** 9915946
- **Project number:** 5R01GM120211-04
- **Recipient organization:** AUBURN UNIVERSITY AT AUBURN
- **Principal Investigator:** Paul A Cobine
- **Activity code:** R01 (R01, R21, SBIR, etc.)
- **Funding institute:** NIH
- **Fiscal year:** 2020
- **Award amount:** $259,481
- **Award type:** 5
- **Project period:** 2017-06-01 → 2022-04-30

## Primary source

NIH RePORTER: https://reporter.nih.gov/project-details/9915946

## Citation

> US National Institutes of Health, RePORTER application 9915946, Copper Transport by Members of the Mitochondrial Carrier Family (5R01GM120211-04). Retrieved via AI Analytics 2026-05-23 from https://api.ai-analytics.org/grant/nih/9915946. Licensed CC0.

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