The fruit fly, Drosophila melanogaster, has proven to be an invaluable model for understanding fundamental physiological processes such as development and aging.
The availability of simple genetic tools that can be used to generate engineered individuals makes this model particularly powerful for elucidating the mechanisms underlying genetic diseases in physiologically relevant systems.
Mitochondrial diseases are a group of currently incurable genetic disorders characterized by dysfunction of the oxidative phosphorylation (OXPHOS) system — the highly conserved energy conversion machinery within mitochondria. The development of D. melanogaster models for mitochondrial diseases began relatively recently but has already provided important insights into the molecular mechanisms and pathological consequences of mitochondrial dysfunction.
Here, we provide an overview of these models and highlight the relevance of D. melanogaster as a valuable system for studying mitochondrial disorders.
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