A Drosophila model of mitochondrial DNA replication: proteins, genes and regulation.
Mitochondrial biogenesis is a critical process in animal development, cellular homeostasis and aging. Mitochondrial DNA replication is an essential part of this process, and both nuclear and mitochondrial DNA mutations are found to result in mitochondrial dysfunction that leads to developmental defects and delays, aging and disease. Drosophila provides an amenable model system to study mitochondrial biogenesis in normal and disease states. This review provides an overview of current approaches to study the proteins involved in mitochondrial DNA replication, the genes that encode them and their regulation. It also presents a survey of cell and animal models under development to mimic the pathophysiology of human mitochondrial disorders.
Garesse
Rafael
R
Departamento de Bioquímica, Instituto de Investigaciones Biomédicas Alberto Sols CSIC-UAM, Facultad de Medicina, Universidad Autónoma de Madrid, Madrid, Spain. rafael.garesse@uam.es
Kaguni
Laurie S
LS
D000818:Animals; D004261:DNA Replication / Q000502:physiology*; D004272:DNA, Mitochondrial / Q000235:genetics / Q000502:physiology*; D004330:Drosophila; D005786:Gene Expression Regulation; D023061:Gene Order; D028361:Mitochondrial Diseases / Q000503:physiopathology*; D024101:Mitochondrial Proteins / Q000235:genetics* / Q000378:metabolism*
D004272:DNA, Mitochondrial; D024101:Mitochondrial Proteins