The Mitochondrial Research Guild Small Grants Program2011-2012 Small Grant Recipients We are pleased to announce we awarded 5 research grants during our 2011-2012 grant cycle. Below is a list of the investigators and grants that were awarded as well as a brief description of each project. Congratulations to these five grant recipients. Enhanced Anesthetic Sensitivity in a Mouse Model of Leigh Syndrome Investigators: Richard Palmiter, Philip Morgan, Margaret Sedensky, & Albert Quintana Amounted Awarded: $50,000 Description: One distinguishing feature of kids with Leigh Syndrome is their sensitivity to anesthetics. We have developed a genetic mouse model the mimics all of the symptoms of Leigh Syndrome - including sensitivity to a common anesthetic, isoflurane. We propose to determine whether the mice are sensitive to other classes of anesthetics and to elucidate the cellular and molecular mechanisms that underlie anesthetic sensitivity. Screening for Novel Components of the Mitochondrial Quality Control System Investigator: Leo Pallanck Amount Awarded: $25,000 Description: Mitochondria are populous cellular constituents that convert food to a chemical form of energy that is used to power the cell. The accumulation of damaged mitochondria is believed to underlie many common aging-related diseases, and the inheritance of damaged mitochondria can result in debilitating neuromuscular disorders. The goal of our work is to identify cellular factors that can selectively detect and eliminate damaged mitochondria such that they can be replaced with healthy mitochondria. Knowledge of these cellular factors could ultimately lead to the development of therapeutic strategies to treat the many diseases in which an accumulation of damaged mitochondria is implicated. Development of a Cost-Effective Next-Generation Sequencing Test for Mitochondrial Disorders and Proficiency Testing for Quality Control. Investigators: Valeria Vasta, Sihoun Hahn, & Kurt Scharfe Amount Awarded: $50,000 Description: Mitochondrial function is maintained by the action of proteins encoded in both nuclear and mitochondrial DNA (mtDNA). Because of this dual genetic control, mitochondrial disease can be caused by mutations in mtDNA or nuclear genes. About 170 nuclear genes have been found to be mutated in patients with mitochondrial dysfunction and are responsible for the majority of pediatric cases. Comprehensive sequencing of all genes already known to be involved in mitochondrial disease would be cost-prohibitive with traditional DNA sequencing technology. We plan to develop a cost-effective test utilizing a new DNA sequencing technology. We believe that this will enable a widespread implementation of this powerful new clinical test for the rapid diagnosis of mitochondrial disease. The additional important value of the test is that, once mutated genes are identified, individualized therapeutic intervention can be more readily devised. Biomarkers of mitochondrial function and defects in vivo Investigator: Kevin Conley Amount Awarded: $25,000 Description: Mitochondria are the powerhouses of the cell. Defects in this powerhouse underlie many maladies, some of which are found in children and can impact normal development. Our goal is to develop methods that diagnose these defects using a clinical MRI machine. Our hope is that early diagnosis using these new methods will identify affected children at a time when treatment can have the maximum benefit. Interaction of Ubiquinone and Complex I Control of Mitochondrial Disease Investigators: Philip Morgan, Margaret Sedensky Amount Awarded: $50,000 Description: Reactive oxygen species (ROS or free radicals) may be produced at an increased rate when the mitochondrial electron transport chain (ETC) is defective. These ROS may lead to further damage of the ETC and begin a downward spiral in the disease state. We intend to determine the effectiveness of exogenous ROS scavengers, such as Coenzyme Q10 (Ubiquinone), in treating mitochondrial disease. The results will help guide the use of these agents in the treatment of patients with defects in different parts of the ETC. If you have any questions about these grants or our program, please contact:
2008-2009 Small Grant Recipients Four grants of $25,000 each:
2007-2008 Small Grant Recipients Three grants of $25,000 each:
For additional information on how you can help please contact Jill Herczog at j.herczog@nwmito-research.org or Ashley Farrington at a.farrington@nwmito-research.org. |
In Loving Memory... Drew Mussey 2008-2011 Justin Brubaker 1997-2009 Mariah Turner 1995-2009 Donald Dee Neuert Click name for PDF Document |
