RRC ID 70706
Author Kowada R, Kodani A, Ida H, Yamaguchi M, Lee IS, Okada Y, Yoshida H.
Title The function of Scox in glial cells is essential for locomotive ability in Drosophila.
Journal Sci Rep
Abstract 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.
Volume 11(1)
Pages 21207
Published 2021-10-27
DOI 10.1038/s41598-021-00663-2
PII 10.1038/s41598-021-00663-2
PMID 34707123
PMC PMC8551190
MeSH Animals Drosophila Proteins* / genetics Drosophila Proteins* / physiology Drosophila melanogaster* / physiology Locomotion* Metallochaperones* / genetics Metallochaperones* / physiology Mitochondria / metabolism Mitochondria / pathology Neuroglia / metabolism* Synapses / metabolism
IF 3.998
Drosophila DGRC#108736 DGRC#112830 DGRC#113173 DGRC#105188 DGRC#105240