Caenorhabditis elegans as a model system for mtDNA replication defects.

Since the isolation and physical characterization of mammalian mitochondrial DNA (mtDNA) over 35 years ago, numerous studies have been conducted in order to understand its structure and properties, including mode of mtDNA replication and transcription. Even today, the mode of mtDNA replication is still a matter of intense debate. We believe that Caenorhabditis elegans holds the promise of identifying molecular mechanisms of mitochondrial replication. C. elegans is a simple and extremely powerful genetic and developmental model system. Their small size, rapid life cycle, the ability to self-fertilize and somatic tissues that consist of post-mitotic cells offer an efficient way to study mitochondrial metabolism. We have recently developed a number of methods in order to study mitochondrial DNA level and mtDNA maintenance during the development of C. elegans. We hope that the techniques described here can assist laboratories interested in understanding modes of mtDNA replication, distribution and mitochondrial morphology in C. elegans.