Mitochondrial Neurodegeneration: Lessons from Drosophila melanogaster Models

The fruit fly-i.e., Drosophila melanogaster-has proven to be a very useful model for the understanding of basic physiological processes, such as development or ageing. The availability of straightforward genetic tools that can be used to produce engineered individuals makes this model extremely interesting for the understanding of the mechanisms underlying genetic diseases in physiological models. Mitochondrial diseases are a group of yet-incurable genetic disorders characterized by the malfunction of the oxidative phosphorylation system (OXPHOS), which is the highly conserved energy transformation system present in mitochondria. The generation of D. melanogaster models of mitochondrial disease started relatively recently but has already provided relevant information about the molecular mechanisms and pathological consequences of mitochondrial dysfunction. Here, we provide an overview of such models and highlight the relevance of D. melanogaster as a model to study mitochondrial disorders.

Automated summary

The fruit fly Drosophila melanogaster is a valuable model organism for studying basic physiological processes and genetic diseases. With the availability of genetic tools, it offers insights into the mechanisms of mitochondrial diseases. Recent development of D. melanogaster models of mitochondrial disease has provided important information on molecular mechanisms and pathological consequences of mitochondrial dysfunction. This article provides an overview of these models and highlights the significance of D. melanogaster as a model for studying mitochondrial disorders.