摘要：Synthesis of cytochrome c oxidase (
Scox) is a
Drosophila homolog of human
SCO2 encoding a metallochaperone that transports copper to cytochrome c, and is an essential protein for the assembly of cytochrome c oxidase in the mitochondrial respiratory chain complex.
SCO2 is highly conserved in a wide variety of species across prokaryotes and eukaryotes, and mutations in
SCO2 are known to cause mitochondrial diseases such as fatal infantile cardioencephalomyopathy, Leigh syndrome, and Charcot-Marie-Tooth disease, a neurodegenerative disorder. These diseases have a common symptom of locomotive dysfunction. However, the mechanisms of their pathogenesis remain unknown, and no fundamental medications or therapies have been established for these diseases. In this study, we demonstrated that the glial cell-specific knockdown of
Scox perturbs the mitochondrial morphology and function, and locomotive behavior in
Drosophila. In addition, the morphology and function of synapses were impaired in the glial cell-specific
Scox knockdown. Furthermore,
Scox knockdown in ensheathing glia, one type of glial cell in
Drosophila, resulted in larval and adult locomotive dysfunction. This study suggests that the impairment of
Scox in glial cells in the
Drosophila CNS mimics the pathological phenotypes observed by mutations in the
SCO2 gene in humans.
