New Disease-Associated Regulators of Mitochondrial Respiration and Stress Response
Abstract
Mitochondrial diseases result from deficiencies in the production of energy and/or from the side effects of trying to produce adequate energy when the machinery for doing so is defective. We have been studying a gene and its protein (MNRR1) that were recently discovered to be needed for energy production in the mitochondria, the place where most of the cell s energy is produced. When we artificially reduce the amount of MNRR1 energy production is reduced and other characteristics of mitochondrial disease are seen. Evidence that we re on the right track comes from the recent discovery that mutations in MNRR1 appear to be the cause of several human neurodegenerative diseases. Therefore, this is a useful model of at least some types of mitochondrial disease and we can harness it for two important tasks: (i) to better understand mitochondrial energy production when oxygen becomes limiting (hypoxia) or cellular energy metabolism otherwise becomes compromised and (ii) to use this model to actually test compounds that might activate MNRR1 and thus restore energy production.
Document Details
- Document Type
- DoD Grant Award
- Publication Date
- Jan 31, 2017
- Source ID
- W81XWH1610516
Entities
People
- Lawrence I. Grossman
Organizations
- United States Army
- Wayne State University