Journal
EXPERIMENTAL CELL RESEARCH
Volume 366, Issue 2, Pages 92-102Publisher
ELSEVIER INC
DOI: 10.1016/j.yexcr.2018.02.035
Keywords
PDHB; Drosophila; Compound eye; Neuron; Neuromuscular junction
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Funding
- Japan Agency for Medical Research and Development (AMED)
- JSPS Core-to-Core Program, Asia-Africa Science Platforms
- JSPS Program [S2802]
- JSPS KAKENHI [16K07346]
- Grants-in-Aid for Scientific Research [17K07500, 16K07346] Funding Source: KAKEN
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Pyruvate dehydrogenase complex deficiency (PDCD) is a common primary cause of defects in mitochondrial function and also can lead to peripheral neuropathy. Pyruvate dehydrogenase El component subunit beta (PDHB) is a subunit of pyruvate dehydrogenase El, which is a well-known component of PDC. In Drosophila melaztogasmr, the CG11876 (dPDHB) gene is a homolog of human PDHB. In this study, we established a Drosophila model with neuron-specific knockdown of dPDHB to investigate its role in neuropathy pathogenesis. Knockdown of dPDHB in pan-neurons induced locomotor defects in both larval and adult stages, which were consistent with abnormal morphology of the motor neuron terminals at neuromuscular junctions and mitochondrial fragmentation in brains. Moreover, neuron-specific knockdown of dPDHB also shortened the lifespan of adult flies. In addition, flies with knockdown of dPDHB manifested a rough eye phenotype and aberrant photoreceptor axon targeting. These results with the Drosophila model suggest the involvement of PDHB in peripheral neuropathy.
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