Drosophila models to study causative genes for human rare intractable neurological diseases

Yamaguchi, Masamitsu; Lee, Im Soon; Jantrapirom, Salinee; Suda, Kojiro; Yoshida, Hideki

doi:10.1016/j.yexcr.2021.112584

Cited by 17 publications

(12 citation statements)

References 205 publications

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“…11 Since about 75% of the genes involved in human pathologies are homologous to Drosophila, this organism can represent a useful model to identify mechanisms associated with different human diseases. 12,13 Despite the above mentioned, it is surprising that few studies have applied the Drosophila model to obtain new insights into the potential hazard resulting from MNPL exposure. Only four recent studies have been found in the literature.…”

Section: Introductionmentioning

confidence: 99%

Hazard assessment of ingested polystyrene nanoplastics in Drosophila larvae

Alaraby

Abass

Domenech

et al. 2022

Environ. Sci.: Nano

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Section: Introductionmentioning

confidence: 99%

Hazard assessment of ingested polystyrene nanoplastics in Drosophila larvae

Alaraby

Abass

Domenech

et al. 2022

Environ. Sci.: Nano

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“…While flies have been widely adopted for studying neurological development, processes and disorders [ 75 , 267 , 268 , 269 ], they have been underutilized as a model for neurological infection. When speed and cost are at a premium, Drosophila models fulfill this need through their ease of genetic manipulation and animal husbandry.…”

Section: Discussionmentioning

confidence: 99%

Drosophila as a Model for Human Viral Neuroinfections

Benoit

Curzio

Civetta

et al. 2022

Cells

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The study of human neurological infection faces many technical and ethical challenges. While not as common as mammalian models, the use of Drosophila (fruit fly) in the investigation of virus–host dynamics is a powerful research tool. In this review, we focus on the benefits and caveats of using Drosophila as a model for neurological infections and neuroimmunity. Through the examination of in vitro, in vivo and transgenic systems, we highlight select examples to illustrate the use of flies for the study of exogenous and endogenous viruses associated with neurological disease. In each case, phenotypes in Drosophila are compared to those in human conditions. In addition, we discuss antiviral drug screening in flies and how investigating virus–host interactions may lead to novel antiviral drug targets. Together, we highlight standardized and reproducible readouts of fly behaviour, motor function and neurodegeneration that permit an accurate assessment of neurological outcomes for the study of viral infection in fly models. Adoption of Drosophila as a valuable model system for neurological infections has and will continue to guide the discovery of many novel virus–host interactions.

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“…Glycolysis affects the maintenance of post-myelinated oligodendrocytes more than mitochondrial respiration, and demyelination disorders resulting from mitochondrial perturbation are linked to increased ROS and aberrant lipid metabolism, but not ATP production 39 – 41 ; therefore, further analysis as to whether our Drosophila model is useful for CMT type 4 is required. Most Drosophila models established thus far for CMT are designed for the axonal type CMT because Drosophila lacks myelin sheaths and Schwan cells 42 . However, the present study suggests that the development of models mimicking some demyelinating type CMT is also possible in Drosophila .…”

Section: Discussionmentioning

confidence: 99%

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

Kowada

Kodani

Ida³

et al. 2021

Sci Rep

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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.

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Drosophila models to study causative genes for human rare intractable neurological diseases

Cited by 17 publications

References 205 publications

Hazard assessment of ingested polystyrene nanoplastics in Drosophila larvae

Hazard assessment of ingested polystyrene nanoplastics in Drosophila larvae

Drosophila as a Model for Human Viral Neuroinfections

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

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