Papers 【 display / non-display 】

Papers 【 display / non-display 】

• Drosophila model to clarify the pathological significance of OPA1 in autosomal dominant optic atrophy

  Yohei Nitta, Jiro Osaka, Ryuto Maki, Satoko Hakeda-Suzuki, Emiko Suzuki, Satoshi Ueki, Takashi Suzu … Show more authors

  eLife   2024.8  [Reviewed]

  DOI

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  Language：English   Publishing type：Research paper (scientific journal)   Single Work  

• Cell adhesion and actin dynamics factors promote axonal extension and synapse formation in transplanted Drosophila photoreceptor cells

  Riku Iwanaga; Nagisa Yahagi; Satoko Hakeda‐Suzuki; Takashi Suzuki

  Development, Growth & Differentiation   2024.2  [Reviewed]

  DOI

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  Language：English   Publishing type：Research paper (scientific journal)   Single Work  

• Complex formation of immunoglobulin superfamily molecules Side-IV and Beat-IIb regulates synaptic specificity

  2024.2  [Reviewed]

  DOI

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  Language：English   Publishing type：Research paper (scientific journal)   Single Work  

• Drosophila model to clarify the pathological significance of OPA1 in autosomal dominant optic atrophy

  Yohei Nitta, Jiro Osaka, Ryuto Maki, Satoko Hakeda-Suzuki, Emiko Suzuki, Satoshi Ueki, Takashi Suzu … Show more authors

  eLife   12   2023.6

  DOI CiNii Research

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  Language：The in addition, foreign language   Publishing type：Research paper (scientific journal)   Publisher：eLife Sciences Publications, Ltd   Joint Work  

  <jats:p>Abstract</jats:p>
  <jats:p>Autosomal dominant optic atrophy (DOA) is a progressive form of blindness caused by degeneration of retinal ganglion cells and their axons, mainly caused by mutations in the OPA1 mitochondrial dynamin like GTPase (OPA1) gene. OPA1 encodes a dynamin-like GTPase present in the mitochondrial inner membrane. When associated with OPA1 mutations, DOA can present not only ocular symptoms but also multi-organ symptoms (DOA plus). DOA plus often results from point mutations in the GTPase domain, which are assumed to have dominant negative effects. However, the presence of mutations in the GTPase domain does not always result in DOA plus. Therefore, an experimental system to distinguish between DOA and DOA plus is needed. In this study, we found that loss-of-function mutations of the dOPA1 gene in Drosophila can imitate the pathology of optic nerve degeneration observed in DOA. We successfully rescued this degeneration by expressing the human OPA1 (hOPA1) gene, indicating that hOPA1 is functionally interchangeable with dOPA1 in the fly system. However, we could not rescue any previously reported mutations known to cause either DOA or DOA plus. By expressing both WT and DOA plus mutant hOPA1 forms in the optic nerve of dOPA1 mutants, we observed that DOA plus mutations suppressed the rescue, facilitating the distinction between loss-of-function and dominant negative mutations in hOPA1. The fly model developed in this study can assist in the differential diagnosis between DOA and DOA plus and inform early treatment decisions in patients with mutations in hOPA1.</jats:p>

• Direct evaluation of neuroaxonal degeneration with the causative genes of neurodegenerative diseases in <i>Drosophila</i> using the automated axon quantification system, MeDUsA

  Nitta, Y; Kawai, H; Maki, R; Osaka, J; Hakeda-Suzuki, S; Nagai, Y; Doubková, K; Uehara, T; Watanabe … Show more authors

  HUMAN MOLECULAR GENETICS   32 ( 9 )   1524 - 1538   2023.4  [Reviewed]

  DOI Web of Science PubMed CiNii Research

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  Language：The in addition, foreign language   Publishing type：Research paper (scientific journal)   Publisher：Oxford University Press (OUP)   Joint Work  

  <jats:title>Abstract</jats:title>
  <jats:p>Drosophila is an excellent model organism for studying human neurodegenerative diseases (NDs). However, there is still almost no experimental system that could directly observe the degeneration of neurons and automatically quantify axonal degeneration. In this study, we created MeDUsA (a ‘method for the quantification of degeneration using fly axons’), a standalone executable computer program based on Python that combines a pre-trained deep-learning masking tool with an axon terminal counting tool. This software automatically quantifies the number of retinal R7 axons in Drosophila from a confocal z-stack image series. Using this software, we were able to directly demonstrate that axons were degenerated by the representative causative genes of NDs for the first time in Drosophila. The fly retinal axon is an excellent experimental system that is capable of mimicking the pathology of axonal degeneration in human NDs. MeDUsA rapidly and accurately quantifies axons in Drosophila photoreceptor neurons. It enables large-scale research into axonal degeneration, including screening to identify genes or drugs that mediate axonal toxicity caused by ND proteins and diagnose the pathological significance of novel variants of human genes in axons.</jats:p>

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Grant-in-Aid for Scientific Research 【 display / non-display 】

Grant-in-Aid for Scientific Research 【 display / non-display 】

• シナプス形成による神経軸索投射と回路網の決定機構

  Grant number：24K09467  2024.4 - 2028.3

  Grant-in-Aid for Scientific Research(C)

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  Authorship：Principal investigator  Grant type：Competitive

• 神経回路の形成における神経間認識の分子機構

  Grant number：21K06184  2021.4 - 2022.4

  Grant-in-Aid for Scientific Research(C)

• Molecular codes which determine the depth of final axonal stabilizing layer in the Drosophila

  Grant number：18K06250  2018.3 - 2021.3

  Grant-in-Aid for Scientific Research(C)

• Temporal control of molecular function in axon wiring of the visual system

  Grant number：26440119  2014.4 - 2017.3

  Grant-in-Aid for Scientific Research(C)

  Investigator(s)：Satoko Hakeda-Suzuki

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  Authorship：Principal investigator  Grant type：Competitive