| RRC ID |
57182
|
| 著者 |
Dung VM, Suong DNA, Okamaoto Y, Hiramatsu Y, Thao DTP, Yoshida H, Takashima H, Yamaguchi M.
|
| タイトル |
Neuron-specific knockdown of Drosophila PDHB induces reduction of lifespan, deficient locomotive ability, abnormal morphology of motor neuron terminals and photoreceptor axon targeting.
|
| ジャーナル |
Exp Cell Res
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| 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.
|
| 巻・号 |
366(2)
|
| ページ |
92-102
|
| 公開日 |
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
|
| 引用数 |
3
|
| リソース情報 |
| ショウジョウバエ |
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