Nuclear Encoded Proteins Important in Mitochondrial Genome Stability

Abstract

The mitochondrion is widely known to be the site of cellular respiration and the factory of cellular energy. Similar to the nucleus, mitochondria house genetic material (mtDNA), which is responsible for the production of proteins essential to mechanisms required for cellular respiration. Furthermore, if there is a mutation or deletion in the mtDNA there can be ramifications in terms of energy production, which will hinder cell viability. Additionally, mutations in the mtDNA are associated with certain neuromuscular diseases as well as contributing to the aging process. The focus of this research is to identify genes that contribute to the maintenance of the mtDNA. Our data from genetic assays indicate that loss of the Clu1p protein exhibits an increase respiration loss as well as increase spontaneous point mutations. In addition, loss of Clu1p alters mitochondrial morphology.