Reference - Detail
| RRC ID | 57182 |
|---|---|
| Author | Dung VM, Suong DNA, Okamaoto Y, Hiramatsu Y, Thao DTP, Yoshida H, Takashima H, Yamaguchi M. |
| Title | Neuron-specific knockdown of Drosophila PDHB induces reduction of lifespan, deficient locomotive ability, abnormal morphology of motor neuron terminals and photoreceptor axon targeting. |
| Journal | Exp Cell Res |
| Abstract |
Pyruvate dehydrogenase complex deficiency (PDCD) is a common primary cause of defects in mitochondrial function and also can lead to peripheral neuropathy. Pyruvate dehydrogenase E1 component subunit beta (PDHB) is a subunit of pyruvate dehydrogenase E1, which is a well-known component of PDC. In Drosophila melanogaster, 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. |
| Volume | 366(2) |
| Pages | 92-102 |
| Published | 2018-5-15 |
| DOI | 10.1016/j.yexcr.2018.02.035 |
| PII | S0014-4827(18)30118-6 |
| PMID | 29501567 |
| MeSH | Animals Animals, Genetically Modified / physiology Axons / metabolism Axons / pathology* Drosophila melanogaster / physiology* Larva / genetics Larva / growth & development Larva / metabolism Locomotion* Longevity* Male Mitochondria / metabolism Mitochondria / pathology Motor Neurons / metabolism Motor Neurons / pathology* Phenotype Photoreceptor Cells, Invertebrate / metabolism Photoreceptor Cells, Invertebrate / pathology* Pyruvate Dehydrogenase (Lipoamide) / antagonists & inhibitors* Pyruvate Dehydrogenase (Lipoamide) / genetics Pyruvate Dehydrogenase (Lipoamide) / metabolism |
| IF | 3.329 |
| Times Cited | 3 |
| Altmetric score |
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| The most frequently cited source | X(Twitter) |
| Total number of mentions | 11 |
| Altmetric score changes over past 6months | 0.0 |
| Resource | |
| Drosophila | |