# Cellular and Molecular Consequences of Respiratory Chain Defects in Neurons

> **NIH NIH R01** · UNIVERSITY OF MIAMI SCHOOL OF MEDICINE · 2023 · $370,690

## Abstract

Defects in mitochondrial oxidative phosphorylation (OXPHOS) have been associated with various primary
mitochondrial diseases as well as with neurodegenerative disorders. Such disorders can be caused by defects in
nuclear or mitochondrial DNA (mtDNA). We propose to take advantage of existing and new mouse models
created in our lab to manipulate the mtDNA to study fundamental mechanisms of the role of mtDNA
mutations in neurodegeneration. We will also use DNA editing enzymes to further modulate mtDNA
heteroplasmy in vivo. We will analyze the ability of different neuronal types (glutamatergic and dopaminergic)
to accumulate mtDNA deletions (aim#1) and a pathogenic point mutation in a tRNA gene (aim#2). With these
models in place, we will also study the susceptibility of glutamatergic and dopaminergic neuronal subtypes to
OXPHOS defects.

## Key facts

- **NIH application ID:** 10680366
- **Project number:** 5R01NS079965-10
- **Recipient organization:** UNIVERSITY OF MIAMI SCHOOL OF MEDICINE
- **Principal Investigator:** Carlos Torres Moraes
- **Activity code:** R01 (R01, R21, SBIR, etc.)
- **Funding institute:** NIH
- **Fiscal year:** 2023
- **Award amount:** $370,690
- **Award type:** 5
- **Project period:** 2012-05-15 → 2025-03-31

## Primary source

NIH RePORTER: https://reporter.nih.gov/project-details/10680366

## Citation

> US National Institutes of Health, RePORTER application 10680366, Cellular and Molecular Consequences of Respiratory Chain Defects in Neurons (5R01NS079965-10). Retrieved via AI Analytics 2026-05-22 from https://api.ai-analytics.org/grant/nih/10680366. Licensed CC0.

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