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Therapeutic reduction of ataxin-2 extends lifespan and reduces pathology in TDP-43 mice

Author

Listed:
  • Lindsay A. Becker

    (Stanford University School of Medicine
    Stanford Neurosciences Graduate Program, Stanford University School of Medicine)

  • Brenda Huang

    (Stanford University School of Medicine)

  • Gregor Bieri

    (Stanford University School of Medicine
    Stanford Neurosciences Graduate Program, Stanford University School of Medicine)

  • Rosanna Ma

    (Stanford University School of Medicine)

  • David A. Knowles

    (Stanford University School of Medicine
    Stanford University School of Medicine, Stanford, California 94305, USA)

  • Paymaan Jafar-Nejad

    (Ionis Pharmaceuticals)

  • James Messing

    (St. Jude Children’s Research Hospital)

  • Hong Joo Kim

    (St. Jude Children’s Research Hospital)

  • Armand Soriano

    (Ionis Pharmaceuticals)

  • Georg Auburger

    (Experimental Neurology, Goethe University)

  • Stefan M. Pulst

    (Experimental Neurology, Goethe University)

  • J. Paul Taylor

    (St. Jude Children’s Research Hospital
    Howard Hughes Medical Institute)

  • Frank Rigo

    (Ionis Pharmaceuticals)

  • Aaron D. Gitler

    (Stanford University School of Medicine)

Abstract

A decrease in ataxin-2 levels leads to a reduction in the aggregation of TDP-43, markedly increased lifespan and improved motor function in a transgenic mouse model of TDP-43 proteinopathy.

Suggested Citation

  • Lindsay A. Becker & Brenda Huang & Gregor Bieri & Rosanna Ma & David A. Knowles & Paymaan Jafar-Nejad & James Messing & Hong Joo Kim & Armand Soriano & Georg Auburger & Stefan M. Pulst & J. Paul Taylo, 2017. "Therapeutic reduction of ataxin-2 extends lifespan and reduces pathology in TDP-43 mice," Nature, Nature, vol. 544(7650), pages 367-371, April.
    • Handle: RePEc:nat:nature:v:544:y:2017:i:7650:d:10.1038_nature22038
    DOI: 10.1038/nature22038
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    Citations

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    Cited by:

    1. Tao Wang & Xibin Tian & Han Byeol Kim & Yura Jang & Zhiyuan Huang & Chan Hyun Na & Jiou Wang, 2022. "Intracellular energy controls dynamics of stress-induced ribonucleoprotein granules," Nature Communications, Nature, vol. 13(1), pages 1-21, December.
    2. M. Alejandra Zeballos C. & Hayden J. Moore & Tyler J. Smith & Jackson E. Powell & Najah S. Ahsan & Sijia Zhang & Thomas Gaj, 2023. "Mitigating a TDP-43 proteinopathy by targeting ataxin-2 using RNA-targeting CRISPR effector proteins," Nature Communications, Nature, vol. 14(1), pages 1-17, December.
    3. Jie Zhong & Chaodong Wang & Dan Zhang & Xiaoli Yao & Quanzhen Zhao & Xusheng Huang & Feng Lin & Chun Xue & Yaqing Wang & Ruojie He & Xu-Ying Li & Qibin Li & Mingbang Wang & Shaoli Zhao & Shabbir Khan , 2024. "PCDHA9 as a candidate gene for amyotrophic lateral sclerosis," Nature Communications, Nature, vol. 15(1), pages 1-22, December.
    4. Renata Vieira de Sá & Emma Sudria-Lopez & Marta Cañizares Luna & Oliver Harschnitz & Dianne M. A. Heuvel & Sandra Kling & Danielle Vonk & Henk-Jan Westeneng & Henk Karst & Lauri Bloemenkamp & Suzy Var, 2024. "ATAXIN-2 intermediate-length polyglutamine expansions elicit ALS-associated metabolic and immune phenotypes," Nature Communications, Nature, vol. 15(1), pages 1-25, December.
    5. Rebecca San Gil & Dana Pascovici & Juliana Venturato & Heledd Brown-Wright & Prachi Mehta & Lidia Madrid San Martin & Jemma Wu & Wei Luan & Yi Kit Chui & Adekunle T. Bademosi & Shilpa Swaminathan & Se, 2024. "A transient protein folding response targets aggregation in the early phase of TDP-43-mediated neurodegeneration," Nature Communications, Nature, vol. 15(1), pages 1-23, December.

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