The function of Scox in glial cells is essential for locomotive ability in Drosophila

• Published in: Scientific reports Vol. 11; no. 1; p. 21207
• Main Authors: Kowada, Ryosuke, Kodani, Atsushi, Ida, Hiroyuki, Yamaguchi, Masamitsu, Lee, Im-Soon, Okada, Yasushi, Yoshida, Hideki
• Format: Journal Article
• Language: English
• Published: London Nature Publishing Group UK 27-10-2021; Nature Publishing Group; Nature Portfolio
• Subjects: 631/136/1425; 631/378/2596; Animals; Charcot-Marie-Tooth disease; Cytochrome; Cytochrome-c oxidase; Cytology; Drosophila; Drosophila melanogaster; Electron transport; Glial cells; Humanities and Social Sciences; Insects; Locomotion; Mitochondria; Mitochondria - metabolism; Mitochondria - pathology; Morphology; multidisciplinary; Mutation; Neurodegenerative diseases; Neuroglia - metabolism; Phenotypes; Prokaryotes; Science; Science (multidisciplinary); Synapses - metabolism
• DOI: 10.1038/s41598-021-00663-2
• EISSN: 2045-2322

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.