Auflistung nach Autor "Hübener-Schmid, Jeannette"

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

Golla, Matthias; Guaitoli, Giambattista; Hayer, Stefanie Nicole; Hauser, Stefan; Krahl, Ann-Christin; Nagel, Maike; Samer, Sebastian; Schöls, Ludger; Weber, Jonasz Jeremiasz; Rieß, Olaf; Gloeckner, Christian; Nguyen, Huu Phuc; Hübener-Schmid, Jeannette (2017)

A Novel SCA3 Knock-in Mouse Model Mimics the Human SCA3 Disease Phenotype Including Neuropathological, Behavioral, and Transcriptional Abnormalities Especially in Oligodendrocytes

Haas, Eva; Incebacak, Rana D.; Hentrich, Thomas; Huridou, Chrisovalantou; Schmidt, Thorsten; Casadei, Nicolas; Maringer, Yacine; Bahl, Carola; Rieß, Olaf; Schulze-Hentrich, Julia Maria; Hübener-Schmid, Jeannette (2022)

A standardised protocol for blood and cerebrospinal fluid collection and processing for biomarker research in ataxia

Ribeiro, Paulo Rogério de Almeida; Hübener-Schmid, Jeannette; Schöls, Ludger; Riess, Olaf (2023)

Allele-specific targeting of mutant ataxin-3 by antisense oligonucleotides in SCA3-iPSC-derived neurons

Hauser, Stefan; Helm, Jacob; Kraft, Melanie; Korneck, Milena; Hübener-Schmid, Jeannette; Schöls, Ludger (2022)

Automated Behavioral Phenotyping Reveals Presymptomatic Alterations in a SCA3 Genetrap Mouse Model

Casadei, Nicolas; Rieß, Olaf; Nguyen, Huu Phuc; Seeliger, Wolfgang Mathias; Hübener-Schmid, Jeannette (2012)

Calpain-1 ablation partially rescues disease-associated hallmarks in models of Machado-Joseph disease

Haas, Eva; Maringer, Yacine; Hauser, Stefan; Weber, Jonasz Jeremiasz; Casadei, Nicolas; Rieß, Olaf; Hübener-Schmid, Jeannette (2020)

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

Hübener-Schmid, Jeannette; Weber, Jonasz Jeremiasz; Richter, Claudia; Honold, Lisa; Murad, Fabronia; Bellstedt, Peter; Paquet-Durand, François; Rieß, Olaf; Nguyen, Huu Phuc (2013)

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

Weber, Jonasz Jeremiasz; Nguyen, Huu Phuc; Hübener-Schmid, Jeannette; Rieß, Olaf (2013)

Characterization of Lifestyle in Spinocerebellar Ataxia Type 3 and Association with Disease Severity

Hengel, Holger; Martus, Peter; Hübener-Schmid, Jeannette; Synofzik, Matthis; Schöls, Ludger (2022)

Development of an AAV-Based MicroRNA Gene Therapy to Treat Machado-Joseph Disease

Stricker-Shaver, Janice; Hübener-Schmid, Jeannette; Haas, Eva (2019)

From Pathways to Targets: Understanding the Mechanisms behind Polyglutamine Disease

Weber, Jonasz Jeremiasz; Sowa, Anna; Binder, Tina; Hübener-Schmid, 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)

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; Hübener-Schmid, Jeannette; Rieß, Olaf (2016)

Increased expression of myelin-associated genes in frontal cortex of SNCA overexpressing rats and Parkinson's disease patients

Hentrich, Thomas; Ehrhardt, Christine; Haas, Eva; Casadei, Nicolas; Outeiro, Tiago F.; Hübener-Schmid, Jeannette; Rieß, Olaf; Schulze-Hentrich, Julia Maria (2020)

Intronic enhancers of the human SNCA gene predominantly regulate its expression in brain in vivo

Cheng, Fubo; Zheng, Wenxu; Yu-Taeger, Libo; Casadei, Nicolas; Admard, Jakob; Sharma, Manu; Grundmann-Hauser, Kathrin; Ott, Thomas; Boldt, Karsten; Junger, Katrin; Ueffing, Marius; Hübener-Schmid, Jeannette; Rieß, Olaf (2022)

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; Schmidt, Jana; Hauser, Stefan; Macek, Boris; Schmidt, Thorsten; Incebacak Eltemur, Rana; Hübener-Schmid, Jeannette; Rieß, Olaf (2022)

Medizinische Zwillingsforschung in Deutschland

Enck, Paul; Goebel-Stengel, Miriam; Kagan, Karl Oliver; Wiesing, Urban; Zipfel, Stephan; Stengel, Andreas; Rieß, Olaf; Hübener-Schmid, Jeannette; Nieß, Andreas Michael; Tümmers, Henning (2021)

Mitochondrial Dysfunction in Spinocerebellar Ataxia Type 3 Is Linked to VDAC1 Deubiquitination

Harmuth, Tina; Zimmer, Anna J.; Schmidt, Jana; Fitzgerald, Julia C.; Weber, Jonasz Jeremiasz; Sowa, Anna; Schöls, Ludger; Rieß, Olaf; Hübener-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 Jeremiasz; Funke, Claudia; Driessen, Stefan; Magg, Janine; Krebiehl, Guido; Wolburg, Hartwig; Hayer, Stefanie Nicole; Hauser, Stefan; Krüger, Rejko; Schöls, Ludger; Rieß, Olaf; Hübener-Schmid, Jeannette (2018)

Model Systems for Spinocerebellar Ataxias: Lessons Learned About the Pathogenesis

Hübener-Schmid, Jeannette; Schmidt, Jana; Schmidt, Thorsten (2012-04-18)

Auflistung nach Autor "Hübener-Schmid, Jeannette"

Auflistung nach Autor "Hübener-Schmid, Jeannette"

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