Powering Up Mitochondrial Functions to Treat Mitochondrial Disease
Abstract
We proposed that induction of the ERRalpha/gamma signaling pathway can enhance mitochondrial function in both cell and animal models of mitochondrial disease. Our major findings include;1) We recently compared in detail the different mitochondrial disease animal models (under review in Cell Metabolism). We found that the compound Ant1-/-ND6 mutant mouse model exhibited the earliest and strongest mitochondrial cardiomyopathy phenotype and therefore provided the best therapeutic window for our proposed intervention research strategy.2) We discovered that GDF15 is a heart-derived hormone whose serum level correlates positively with the severity of mitochondrial cardiomyopathy (recently published with DOD grant support acknowledged), and it can be used as a biomarker in our studies.
Document Details
- Document Type
- Technical Report
- Publication Date
- Oct 01, 2017
- Accession Number
- AD1048403
Entities
People
- Douglas C Wallace
- Liming Pei