| Calpain-mediated ataxin-3 cleavage in the molecular pathogenesis of spinocerebellar ataxia type 3 (SCA3) J Hübener, JJ Weber, C Richter, L Honold, A Weiss, F Murad, P Breuer, ... Human molecular genetics 22 (3), 508-518, 2013 | 115 | 2013 |
| From pathways to targets: understanding the mechanisms behind polyglutamine disease JJ Weber, AS Sowa, T Binder, J Hübener BioMed research international 2014, 2014 | 76 | 2014 |
| Karyopherin α-3 is a key protein in the pathogenesis of spinocerebellar ataxia type 3 controlling the nuclear localization of ataxin-3 AS Sowa, E Martin, IM Martins, J Schmidt, R Depping, JJ Weber, F Rother, ... Proceedings of the National Academy of Sciences 115 (11), E2624-E2633, 2018 | 47 | 2018 |
| A combinatorial approach to identify calpain cleavage sites in the Machado-Joseph disease protein ataxin-3 JJ Weber, M Golla, G Guaitoli, P Wanichawan, SN Hayer, S Hauser, ... Brain 140 (5), 1280-1299, 2017 | 46 | 2017 |
| Olesoxime suppresses calpain activation and mutant huntingtin fragmentation in the BACHD rat LE Clemens, JJ Weber, TT Wlodkowski, L Yu-Taeger, M Michaud, ... Brain 138 (12), 3632-3653, 2015 | 45 | 2015 |
| Mitochondrial morphology, function and homeostasis are impaired by expression of an N-terminal calpain cleavage fragment of ataxin-3 T Harmuth, C Prell-Schicker, JJ Weber, F Gellerich, C Funke, S Drießen, ... Frontiers in molecular neuroscience 11, 368, 2018 | 39 | 2018 |
| In vivo assessment of riluzole as a potential therapeutic drug for spinocerebellar ataxia type 3 J Schmidt, T Schmidt, M Golla, L Lehmann, JJ Weber, J Hübener‐Schmid, ... Journal of Neurochemistry 138 (1), 150-162, 2016 | 37 | 2016 |
| Olesoxime in neurodegenerative diseases: Scrutinising a promising drug candidate JJ Weber, LE Clemensson, HB Schiöth, HP Nguyen Biochemical Pharmacology 168, 305-318, 2019 | 33 | 2019 |
| Cerebellar soluble mutant ataxin-3 level decreases during disease progression in Spinocerebellar Ataxia Type 3 mice HP Nguyen, J Hübener, JJ Weber, S Grueninger, O Riess, A Weiss PLoS One 8 (4), e62043, 2013 | 28 | 2013 |
| Calpastatin ablation aggravates the molecular phenotype in cell and animal models of Huntington disease JJ Weber, SJ Kloock, M Nagel, MM Ortiz-Rios, J Hofmann, O Riess, ... Neuropharmacology 133, 94-106, 2018 | 25 | 2018 |
| Reduced cell size, chromosomal aberration and altered proliferation rates are characteristics and confounding factors in the STHdh cell model of Huntington disease E Singer, C Walter, JJ Weber, AC Krahl, UA Mau-Holzmann, N Rischert, ... Scientific reports 7 (1), 16880, 2017 | 24 | 2017 |
| Killing two angry birds with one stone: autophagy activation by inhibiting calpains in neurodegenerative diseases and beyond JJ Weber, P Pereira Sena, E Singer, HP Nguyen BioMed Research International 2019, 2019 | 21 | 2019 |
| The calpain-suppressing effects of olesoxime in Huntington's disease JJ Weber, MM Ortiz Rios, O Riess, LE Clemens, HP Nguyen Rare Diseases 4 (1), e1153778, 2016 | 20 | 2016 |
| Calpain-1 ablation partially rescues disease-associated hallmarks in models of Machado-Joseph disease JJ Weber, E Haas, Y Maringer, S Hauser, NLP Casadei, AH Chishti, ... Human Molecular Genetics 29 (6), 892-906, 2020 | 17 | 2020 |
| Environment-dependent striatal gene expression in the BACHD rat model for Huntington disease A Novati, T Hentrich, Z Wassouf, JJ Weber, L Yu-Taeger, N Déglon, ... Scientific reports 8 (1), 5803, 2018 | 13 | 2018 |
| Mitochondrial dysfunction in spinocerebellar ataxia type 3 is linked to VDAC1 deubiquitination T Harmuth, JJ Weber, AJ Zimmer, AS Sowa, J Schmidt, JC Fitzgerald, ... International Journal of Molecular Sciences 23 (11), 5933, 2022 | 10 | 2022 |
| Generation of an induced pluripotent stem cell line from a patient with spinocerebellar ataxia type 3 (SCA3): HIHCNi002-A SN Hayer, Y Schelling, J Huebener-Schmid, JJ Weber, S Hauser, ... Stem cell research 30, 171-174, 2018 | 10 | 2018 |
| Vulnerability of frontal brain neurons for the toxicity of expanded ataxin-3 J Schmidt, AK Mayer, D Bakula, J Freude, JJ Weber, A Weiss, O Riess, ... Human Molecular Genetics 28 (9), 1463-1473, 2019 | 9 | 2019 |
| Pathophysiological interplay between O-GlcNAc transferase and the Machado–Joseph disease protein ataxin-3 P Pereira Sena, JJ Weber, M Watchon, KJ Robinson, Z Wassouf, ... Proceedings of the National Academy of Sciences 118 (47), e2025810118, 2021 | 7 | 2021 |
| Calpain-mediated proteolysis as driver and modulator of polyglutamine toxicity RD Incebacak Eltemur, HP Nguyen, JJ Weber Frontiers in Molecular Neuroscience 15, 1020104, 2022 | 6 | 2022 |
