Powering Up Mitichondrial Functions to Treat Mitochondrial Disease
Abstract
We proposed that induction of the ERRalpha/gamma signaling pathway can enhance mitochondrial functionin 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
- AD1048404
Entities
People
- Douglas C Wallace
- Liming Pei