The University Hospital

Dr. rer. nat. Jonasz Jeremiasz Weber

Dr. rer. nat. Jonasz Jeremiasz Weber

Group Leader - Neurodegradomics Research Group (at the Ruhr University Bochum)

As part of my scientific research, I investigate molecular pathomechanisms underlying neurodegenerative diseases. My focus is on enzyme-mediated proteolytic processes in the brain and their evaluation as potential therapeutic targets.

Contact

Phone number: Office: +49 7071 29-72257

E-mail address: jonasz.weber@med.uni-tuebingen.de

Clinic / Institute / Center

  • Molecular and cellular neurobiology
  • Investigation of proteolytic processes as pathomechanisms and biomarkers of neurodegenerative diseases
  • Identification of potenial therapeutic targets for treating rare neurodegenerative diseases
  • since 2021
    Group leader - Neurodegradomics work group, Department of Human Genetics, Ruhr University Bochum, Bochum
  • 2020
    PostDoc at the Department of Human Genetics, Ruhr University Bochum, Bochum
  • since 2017
    PostDoc at the Institute of Medical Genetics and Applied Genomics, University of Tübingen, Tübingen
  • 2011 - 2017
    PhD thesis at the Institute of Medical Genetics and Applied Genomics, University of Tübingen, Tübingen
  • 2005 - 2011
    Studies in Biology at the Eberhard Karls University, Tübingen
  • 2025 - Editorial Board Award 2025, Frontiers in Molecular Neuroscience
  • 2022 - Murachi Postdoctoral Award, FASEB SRC - The Biology of Calpains in Health and Disease Conference
  • 2021 - Medical advancement award of the Sophia & Fritz Heinemann Foundation
  • 2019 - Travel grant, German Society of Human Genetics (GfH)
  • Weber, J. J.*, Czisch, L.*, Pereira Sena, P., Fath, F., Huridou, C., Schwarz, N., Incebacak Eltemur, R. D., Würth, A., Weishäupl, D., Döcker, M., Blumenstock, G., Martins, S., Sequeiros, J., Rouleau, G. A., Jardim, L. B., Saraiva-Pereira, M. L., França, M. C., Jr, Gordon, C. R., Zaltzman, R., Cornejo-Olivas, M. R., … Schmidt, T. (2024). The parkin V380L variant is a genetic modifier of Machado-Joseph disease with impact on mitophagy. Acta neuropathologica, 148(1), 14. https://doi.org/10.1007/s00401-024-02762-6
  • Abeditashi, M., Weber, J. J., Pereira Sena, P., Velic, A., Kalimeri, M., Incebacak Eltemur, R. D., Schmidt, J., Hübener-Schmid, J., Hauser, S., Macek, B., Riess, O., & Schmidt, T. (2022). KPNB1 modulates the Machado-Joseph disease protein ataxin-3 through activation of the mitochondrial protease CLPP. Cellular and molecular life sciences : CMLS, 79(8), 401. https://doi.org/10.1007/s00018-022-04372-5
  • Weber, J. J.*, Anger, S. C.*, Pereira Sena, P., Incebacak Eltemur, R. D., Huridou, C., Fath, F., Gross, C., Casadei, N., Riess, O., & Nguyen, H. P. (2022). Calpains as novel players in the molecular pathogenesis of spinocerebellar ataxia type 17. Cellular and molecular life sciences : CMLS, 79(5), 262. https://doi.org/10.1007/s00018-022-04274-6
  • Pereira Sena, P., Weber, J. J., Watchon, M., Robinson, K. J., Wassouf, Z., Hauser, S., Helm, J., Abeditashi, M., Schmidt, J., Hübener-Schmid, J., Schöls, L., Laird, A. S., Riess, O., & Schmidt, T. (2021). Pathophysiological interplay between O-GlcNAc transferase and the Machado-Joseph disease protein ataxin-3. Proceedings of the National Academy of Sciences of the United States of America, 118(47), e2025810118. https://doi.org/10.1073/pnas.2025810118
  • Sowa, A. S., Martin, E., Martins, I. M., Schmidt, J., Depping, R., Weber, J. J., Rother, F., Hartmann, E., Bader, M., Riess, O., Tricoire, H., & Schmidt, T. (2018). Karyopherin α-3 is a key protein in the pathogenesis of spinocerebellar ataxia type 3 controlling the nuclear localization of ataxin-3. Proceedings of the National Academy of Sciences of the United States of America, 115(11), E2624–E2633. https://doi.org/10.1073/pnas.1716071115
  • Weber, J. J., Golla, M., Guaitoli, G., Wanichawan, P., Hayer, S. N., Hauser, S., Krahl, A. C., Nagel, M., Samer, S., Aronica, E., Carlson, C. R., Schöls, L., Riess, O., Gloeckner, C. J., Nguyen, H. P., & Hübener-Schmid, J. (2017). A combinatorial approach to identify calpain cleavage sites in the Machado-Joseph disease protein ataxin-3. Brain : a journal of neurology, 140(5), 1280–1299. https://doi.org/10.1093/brain/awx039
  • Clemens, L. E.*, Weber, J. J.*, Wlodkowski, T. T., Yu-Taeger, L., Michaud, M., Calaminus, C., Eckert, S. H., Gaca, J., Weiss, A., Magg, J. C., Jansson, E. K., Eckert, G. P., Pichler, B. J., Bordet, T., Pruss, R. M., Riess, O., & Nguyen, H. P. (2015). Olesoxime suppresses calpain activation and mutant huntingtin fragmentation in the BACHD rat. Brain : a journal of neurology, 138(Pt 12), 3632–3653. https://doi.org/10.1093/brain/awv290
  • Hübener, J.*, Weber, J. J.*, Richter, C., Honold, L., Weiss, A., Murad, F., Breuer, P., Wüllner, U., Bellstedt, P., Paquet-Durand, F., Takano, J., Saido, T. C., Riess, O., & Nguyen, H. P. (2013). Calpain-mediated ataxin-3 cleavage in the molecular pathogenesis of spinocerebellar ataxia type 3 (SCA3). Human molecular genetics, 22(3), 508–518. https://doi.org/10.1093/hmg/dds449
  • * shared authorships
  • A full list of publications is available here.