# Examining Transsulfuration in Mitochondrial Hepatopathy in vivo

> **NIH NIH F31** · UT SOUTHWESTERN MEDICAL CENTER · 2021 · $14,961

## Abstract

PROJECT SUMMARY/ABSTRACT
Mitochondrial diseases are a heterogenous class of metabolic disorders affecting 1:5000 people with poor
prognosis and no effective treatment options. The underlying mechanisms of organ-specific phenotypes in
mitochondrial disease are still unknown. In patient-derived cell lines, glucose is re-routed towards lactate
production, and lactic acidosis is a feature of mitochondrial diseases. Metabolic reprogramming of one carbon
metabolism, specifically an increase in transsulfuration genes has been identified; however, these results have
not been investigated in vivo. This proposal aims to understand how transsulfuration serves as a
compensatory mechanism in mitochondrial hepatopathy. To accomplish this, stable isotope tracing mass
spectrometry and mouse models of mitochondrial hepatopathy will be utilized. The specific aims of this
proposal intend to (1) identify if transsulfuration is remodeled in hepatic mitochondrial dysfunction, (2) test how
transsulfuration activity affects liver metabolism and redox status, and (3) determine how cysteine-stimulated
transsulfuration promotes glucose metabolism. The findings of this proposal will provide insights into the
metabolic reprogramming of a large class of metabolic diseases.

## Key facts

- **NIH application ID:** 10119149
- **Project number:** 5F31DK122676-02
- **Recipient organization:** UT SOUTHWESTERN MEDICAL CENTER
- **Principal Investigator:** Nicholas Paul Lesner
- **Activity code:** F31 (R01, R21, SBIR, etc.)
- **Funding institute:** NIH
- **Fiscal year:** 2021
- **Award amount:** $14,961
- **Award type:** 5
- **Project period:** 2020-04-01 → 2021-08-02

## Primary source

NIH RePORTER: https://reporter.nih.gov/project-details/10119149

## Citation

> US National Institutes of Health, RePORTER application 10119149, Examining Transsulfuration in Mitochondrial Hepatopathy in vivo (5F31DK122676-02). Retrieved via AI Analytics 2026-05-22 from https://api.ai-analytics.org/grant/nih/10119149. Licensed CC0.

---

*[NIH grants dataset](/datasets/nih-grants) · CC0 1.0*