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Direct evaluation of neuroaxonal degeneration with the causative genes of neurodegenerative diseases in Drosophila using the automated axon quantification system, MeDUsA.
Nitta, Yohei; Kawai, Hiroki; Maki, Ryuto; Osaka, Jiro; Hakeda-Suzuki, Satoko; Nagai, Yoshitaka; Doubková, Karolína; Uehara, Tomoko; Watanabe, Kenji; Kosaki, Kenjiro; Suzuki, Takashi; Tavosanis, Gaia; Sugie, Atsushi.
Afiliación
  • Nitta Y; Brain Research Institute, Niigata University, Niigata 951-8585, Japan.
  • Kawai H; LPIXEL Inc., Tokyo 100-0004, Japan.
  • Maki R; School of Life Science and Technology, Tokyo Institute of Technology, Yokohama 226-8501, Japan.
  • Osaka J; School of Life Science and Technology, Tokyo Institute of Technology, Yokohama 226-8501, Japan.
  • Hakeda-Suzuki S; School of Life Science and Technology, Tokyo Institute of Technology, Yokohama 226-8501, Japan.
  • Nagai Y; Department of Neurotherapeutics, Osaka University Graduate School of Medicine, Suita 565-0871, Japan.
  • Doubková K; Department of Neurology, Kindai University Faculty of Medicine, Osaka-Sayama 589-8511, Japan.
  • Uehara T; German Center for Neurodegenerative Diseases (DZNE), Bonn 53127, Germany.
  • Watanabe K; RWTH Aachen Institut für Entwicklungsbiologie, Worringerweg Aachen 352074, Germany.
  • Kosaki K; Division of Clinical Genetics, Aichi Developmental Disability Center Hospital, Kasugai 480-0392, Japan.
  • Suzuki T; Department of Pediatrics, Kagoshima City Hospital, Kagoshima 890-8760, Japan.
  • Tavosanis G; Center for Medical Genetics, Keio University School of Medicine, Tokyo 160-8582, Japan.
  • Sugie A; School of Life Science and Technology, Tokyo Institute of Technology, Yokohama 226-8501, Japan.
Hum Mol Genet ; 32(9): 1524-1538, 2023 04 20.
Article en En | MEDLINE | ID: mdl-36611008
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.
Asunto(s)

Texto completo: 1 Colección: 01-internacional Base de datos: MEDLINE Asunto principal: Enfermedades Neurodegenerativas / Proteínas de Drosophila Límite: Animals / Humans Idioma: En Revista: Hum Mol Genet Asunto de la revista: BIOLOGIA MOLECULAR / GENETICA MEDICA Año: 2023 Tipo del documento: Article País de afiliación: Japón Pais de publicación: Reino Unido

Texto completo: 1 Colección: 01-internacional Base de datos: MEDLINE Asunto principal: Enfermedades Neurodegenerativas / Proteínas de Drosophila Límite: Animals / Humans Idioma: En Revista: Hum Mol Genet Asunto de la revista: BIOLOGIA MOLECULAR / GENETICA MEDICA Año: 2023 Tipo del documento: Article País de afiliación: Japón Pais de publicación: Reino Unido