Article
A Drosophila model of mitochondrial disease caused by a complex I mutation that uncouples proton pumping from electron transfer.
Disease models & mechanisms - 1 Oct 2014
Burman Jonathon L, Itsara Leslie S, Kayser Ernst-Bernhard, Suthammarak Wichit, Wang Adrienne M, Kaeberlein Matt, Sedensky Margaret M, Morgan Philip G, Pallanck Leo J
Abstract excerpt
Mutations affecting mitochondrial complex I, a multi-subunit assembly that couples electron transfer to proton pumping, are the most frequent cause of heritable mitochondrial diseases. However, the mechanisms by which complex I dysfunction results in disease remain unclear. Here, we describe a Drosophila model of complex I deficiency caused by a homoplasmic mutation in the mitochondrial-DNA-encoded NADH...
Topics
- Animals
- Disease Models, Animal
- Drosophila
- Electron Transport
- Electron Transport Complex I
- Mitochondrial Diseases
- Mutation
- Oxidative Phosphorylation
- Proton Pumps
- Reactive Oxygen Species