All Stories

  1. Killing Two Angry Birds with One Stone: Autophagy Activation by Inhibiting Calpains in Neurodegenerative Diseases and Beyond

    Article • BioMed Research International, February 2019, Hindawi Publishing Corporation

    Dr. Jonasz Jeremiasz Weber, Priscila Pereira Sena

  2. Vulnerability of frontal brain neurons for the toxicity of expanded ataxin-3

    Article • Human Molecular Genetics, December 2018, Oxford University Press (OUP)

    Dr. Jonasz Jeremiasz Weber

  3. Mitochondrial Morphology, Function and Homeostasis Are Impaired by Expression of an N-terminal Calpain Cleavage Fragment of Ataxin-3

    Article • Frontiers in Molecular Neuroscience, October 2018, Frontiers

    Dr. Jonasz Jeremiasz Weber

  4. Generation of an induced pluripotent stem cell line from a patient with spinocerebellar ataxia type 3 (SCA3): HIHCNi002-A

    Article • Stem Cell Research, July 2018, Elsevier

    Dr. Jonasz Jeremiasz Weber

  5. Calpastatin ablation aggravates the molecular phenotype in cell and animal models of Huntington disease

    Article • Neuropharmacology, May 2018, Elsevier

    Dr. Jonasz Jeremiasz Weber

  6. Environment-dependent striatal gene expression in the BACHD rat model for Huntington disease

    Article • Scientific Reports, April 2018, Springer Science + Business Media

    Professor Nicole Déglon, Dr. Jonasz Jeremiasz Weber

  7. Karyopherin α-3 is a key protein in the pathogenesis of spinocerebellar ataxia type 3 controlling the nuclear localization of ataxin-3

    Article • Proceedings of the National Academy of Sciences, February 2018, Proceedings of the National Academy of Sciences

    Dr. Jonasz Jeremiasz Weber

  8. Reduced cell size, chromosomal aberration and altered proliferation rates are characteristics and confounding factors in the STHdh cell model of Huntington disease

    Article • Scientific Reports, December 2017, Springer Science + Business Media

    Dr. Jonasz Jeremiasz Weber

  9. A combinatorial approach to identify calpain cleavage sites in the Machado-Joseph disease protein ataxin-3

    Article • Brain, March 2017, Oxford University Press (OUP)

    Dr. Jonasz Jeremiasz Weber

  10. B46 The HD-like type 4/SCA17 disease protein TBP is cleaved by calpains in vitro and in vivo

    Article • Journal of Neurology Neurosurgery & Psychiatry, September 2016, BMJ

    Dr. Jonasz Jeremiasz Weber

  11. C3 Reduced cell size and enhanced cell proliferation are characteristics of sthdhq111/111 cells and should be considered as possible confounding factors

    Article • Journal of Neurology Neurosurgery & Psychiatry, September 2016, BMJ

    Dr. Jonasz Jeremiasz Weber

  12. L20 Olesoxime targets calpain overactivation in models of huntington’s disease

    Article • Journal of Neurology Neurosurgery & Psychiatry, September 2016, BMJ

    Dr. Jonasz Jeremiasz Weber

  13. In vivoassessment of riluzole as a potential therapeutic drug for spinocerebellar ataxia type 3

    Article • Journal of Neurochemistry, June 2016, Wiley

    Dr. Jonasz Jeremiasz Weber

  14. The calpain-suppressing effects of olesoxime in Huntington's disease

    Article • Rare Diseases, January 2016, Taylor & Francis

    Dr. Jonasz Jeremiasz Weber

  15. Olesoxime suppresses calpain activation and mutant huntingtin fragmentation in the BACHD rat

    Article • Brain, October 2015, Oxford University Press (OUP)

    Dr. Jonasz Jeremiasz Weber

  16. B05 New Light On The Role Of Calpain-mediated Proteolysis Of Mutant Huntingtin

    Article • Journal of Neurology Neurosurgery & Psychiatry, September 2014, BMJ

    Dr. Jonasz Jeremiasz Weber

  17. From Pathways to Targets: Understanding the Mechanisms behind Polyglutamine Disease

    Article • BioMed Research International, January 2014, Hindawi Publishing Corporation

    Dr. Jonasz Jeremiasz Weber

  18. Cerebellar Soluble Mutant Ataxin-3 Level Decreases during Disease Progression in Spinocerebellar Ataxia Type 3 Mice

    Article • PLoS ONE, April 2013, PLOS

    Dr. Jonasz Jeremiasz Weber

  19. Calpain-mediated ataxin-3 cleavage in the molecular pathogenesis of spinocerebellar ataxia type 3 (SCA3)

    Article • Human Molecular Genetics, October 2012, Oxford University Press (OUP)

    Dr. Jonasz Jeremiasz Weber