Post-transcriptional Silencing and Functional Characterization of the Drosophila melanogaster Homolog of Human Surf1 (original) (raw)
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Division of Molecular Neurogenetics
, National Institute of Neurology “C. Besta,” Milano, Italy and
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CNR Institute of Biomedical Technology
, University of Padova, Padova, Italy
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Accepted:
15 September 2005
Published:
01 January 2006
Cite
Mauro A Zordan, Paola Cisotto, Clara Benna, Alessandro Agostino, Giorgia Rizzo, Alberto Piccin, Mirko Pegoraro, Federica Sandrelli, Giuliana Perini, Giuseppe Tognon, Raffaele De Caro, Samantha Peron, Truus te Kronniè, Aram Megighian, Carlo Reggiani, Massimo Zeviani, Rodolfo Costa, Post-transcriptional Silencing and Functional Characterization of the Drosophila melanogaster Homolog of Human Surf1, Genetics, Volume 172, Issue 1, 1 January 2006, Pages 229–241, https://doi.org/10.1534/genetics.105.049072
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Abstract
Mutations in Surf1, a human gene involved in the assembly of cytochrome c oxidase (COX), cause Leigh syndrome, the most common infantile mitochondrial encephalopathy, characterized by a specific COX deficiency. We report the generation and characterization of functional knockdown (KD) lines for Surf1 in Drosophila. KD was produced by post-transcriptional silencing employing a transgene encoding a dsRNA fragment of the Drosophila homolog of human Surf1, activated by the UAS transcriptional activator. Two alternative drivers, Actin5C_–_GAL4 or elav_–_GAL4, were used to induce silencing ubiquitously or in the CNS, respectively. Actin5C–GAL4 KD produced 100% egg-to-adult lethality. Most individuals died as larvae, which were sluggish and small. The few larvae reaching the pupal stage died as early imagos. Electron microscopy of larval muscles showed severely altered mitochondria. _elav–GAL4_-driven KD individuals developed to adulthood, although cephalic sections revealed low COX-specific activity. Behavioral and electrophysiological abnormalities were detected, including reduced photoresponsiveness in KD larvae using either driver, reduced locomotor speed in Actin5C–GAL4 KD larvae, and impaired optomotor response as well as abnormal electroretinograms in elav–GAL4 KD flies. These results indicate important functions for SURF1 specifically related to COX activity and suggest a crucial role of mitochondrial energy pathways in organogenesis and CNS development and function.
© Genetics 2006
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