Browsing Universitätsbibliographie by Author "Weber, Jonasz Jeremiasz"

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

Weber, Jonasz J.; Golla, Matthias; Guaitoli, Giambattista; Wanichawan, Pimthanya; Hayer, Stefanie N.; Hauser, Stefan; Krahl, Ann-Christin; Nagel, Maike; Samer, Sebastian; Aronica, Eleonora; Carlson, Cathrine R.; Schoels, Ludger; Riess, Olaf; Gloeckner, Christian J.; Nguyen, Huu P.; Huebener-Schmid, Jeannette (2017)
Calpain-1 ablation partially rescues disease-associated hallmarks in models of Machado-Joseph disease

Weber, Jonasz J.; Haas, Eva; Maringer, Yacine; Hauser, Stefan; Casadei, Nicolas L. P.; Chishti, Athar H.; Riess, Olaf; Huebener-Schmid, Jeannette (2020)
Calpain-mediated ataxin-3 cleavage in the molecular pathogenesis of spinocerebellar ataxia type 3 (SCA3)

Huebener, Jeannette; Weber, Jonasz Jeremiasz; Richter, Claudia; Honold, Lisa; Weiss, Andreas; Murad, Fabronia; Breuer, Peter; Wuellner, Ullrich; Bellstedt, Peter; Paquet-Durand, Francois; Takano, Jiro; Saido, Takaomi C.; Riess, Olaf; Huu Phuc Nguyen (2013)
Calpain-mediated proteolysis as driver and modulator of polyglutamine toxicity

Eltemur, Rana Dilara Incebacak; Nguyen, Huu Phuc; Weber, Jonasz Jeremiasz (2022)
Calpains as novel players in the molecular pathogenesis of spinocerebellar ataxia type 17

Weber, Jonasz Jeremiasz; Anger, Stefanie Cari; Sena, Priscila Pereira; Eltemur, Rana Dilara Incebacak; Huridou, Chrisovalantou; Fath, Florian; Gross, Caspar; Casadei, Nicolas; Riess, Olaf; Huu Phuc Nguyen (2022)
Calpains in the molecular pathogenesis of polyglutamine disorders and Their potential as a therapeutic target

Weber, Jonasz Jeremiasz (2017)
Calpastatin ablation aggravates the molecular phenotype in cell and animal models of Huntington disease

Weber, Jonasz Jeremiasz; Kloock, Simon Johannes; Nagel, Maike; Ortiz-Rios, Midea Malena; Hofmann, Julian; Riess, Olaf; Huu Phuc Nguyen (2018)
Cerebellar Soluble Mutant Ataxin-3 Level Decreases during Disease Progression in Spinocerebellar Ataxia Type 3 Mice

Huu Phuc Nguyen; Huebener, Jeannette; Weber, Jonasz Jeremiasz; Grueninger, Stephan; Riess, Olaf; Weiss, Andreas (2013)
Environment-dependent striatal gene expression in the BACHD rat model for Huntington disease

Novati, Arianna; Hentrich, Thomas; Wassouf, Zinah; Weber, Jonasz J.; Deglon, Nicole; Huu Phuc Nguyen; Yu-Taeger, Libo; Schulze-Hentrich, Julia (2018)
Evidences for Mutant Huntingtin Inducing Musculoskeletal and Brain Growth Impairments via Disturbing Testosterone Biosynthesis in Male Huntington Disease Animals

Yu-Taeger, Libo; Novati, Arianna; Weber, Jonasz Jeremiasz; Singer-Mikosch, Elisabeth; Pabst, Ann-Sophie; Cheng, Fubo; Saft, Carsten; Koenig, Jennifer; Ellrichmann, Gisa; Heikkinen, Taneli; Pouladi, Mahmoud A.; Riess, Olaf; Nguyen, Huu Phuc (2022)
From Pathways to Targets: Understanding the Mechanisms behind Polyglutamine Disease

Weber, Jonasz Jeremiasz; Sowa, Anna Sergeevna; Binder, Tina; Huebener, Jeannette (2014)
Generation of an induced pluripotent stem cell line from a patient with spinocerebellar ataxia type 3 (SCA3): HIHCNi002-A

Hayer, Stefanie Nicole; Schelling, Yvonne; Hübener-Schmid, Jeannette; Weber, Jonasz Jeremiasz; Hauser, Stefan; Schöls, Ludger (2018)
Implications of specific lysine residues within ataxin-3 for the molecular pathogenesis of Machado-Joseph disease

Sena, Priscila Pereira; Weber, Jonasz Jeremiasz; Bayezit, Sercan; Saup, Rafael; Eltemur, Rana Dilara Incebacak; Li, Xiaoling; Velic, Ana; Jung, Jaqueline; Macek, Boris; Nguyen, Huu Phuc; Riess, Olaf; Schmidt, Thorsten (2023)
In vivo assessment of riluzole as a potential therapeutic drug for spinocerebellar ataxia type 3

Schmidt, Jana; Schmidt, Thorsten; Golla, Matthias; Lehmann, Lisa; Weber, Jonasz Jeremiasz; Huebener-Schmid, Jeannette; Riess, Olaf (2016)
Karyopherin alpha-3 is a key protein in the pathogenesis of spinocerebellar ataxia type 3 controlling the nuclear localization of ataxin-3

Sowa, Anna Sergeevna; Martin, Elodie; Martins, Ines Morgado; Schmidt, Jana; Depping, Reinhard; Weber, Jonasz Jeremiasz; Rother, Franziska; Hartmann, Enno; Bader, Michael; Riess, Olaf; Tricoire, Herve; Schmidt, Thorsten (2018)
Killing Two Angry Birds with One Stone : Autophagy Activation by Inhibiting Calpains in Neurodegenerative Diseases and Beyond

Weber, Jonasz Jeremiasz; Sena, Priscila Pereira; Singer, Elisabeth; Huu Phuc Nguyen (2019)
KPNB1 modulates the Machado-Joseph disease protein ataxin-3 through activation of the mitochondrial protease CLPP

Abeditashi, Mahkameh; Weber, Jonasz Jeremiasz; Sena, Priscila Pereira; Velic, Ana; Kalimeri, Maria; Eltemur, Rana Dilara Incebacak; Schmidt, Jana; Huebener-Schmid, Jeannette; Hauser, Stefan; Macek, Boris; Riess, Olaf; Schmidt, Thorsten (2022)
Mitochondrial Dysfunction in Spinocerebellar Ataxia Type 3 Is Linked to VDAC1 Deubiquitination

Harmuth, Tina; Weber, Jonasz J.; Zimmer, Anna J.; Sowa, Anna S.; Schmidt, Jana; Fitzgerald, Julia C.; Schoels, Ludger; Riess, Olaf; Huebener-Schmid, Jeannette (2022)
Mitochondrial Morphology, Function and Homeostasis Are Impaired by Expression of an N-terminal Calpain Cleavage Fragment of Ataxin-3

Harmuth, Tina; Prell-Schicker, Caroline; Weber, Jonasz J.; Gellerich, Frank; Funke, Claudia; Driessen, Stefan; Magg, Janine C. D.; Krebiehl, Guido; Wolburg, Hartwig; Hayer, Stefanie N.; Hauser, Stefan; Krueger, Rejko; Schoels, Ludger; Riess, Olaf; Huebener-Schmid, Jeannette (2018)
Neurodegenerative phosphoprotein signaling landscape in models of SCA3

Sowa, Anna S.; Popova, Taissia G.; Harmuth, Tina; Weber, Jonasz J.; Pereira Sena, Priscila; Schmidt, Jana; Huebener-Schmid, Jeannette; Schmidt, Thorsten (2021)

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