Mid-Gestation lethality of <i>Atxn2l</i>-Ablated Mice

by Jana Key, Patrick N. Harter, Nesli-Ece Sen, Elise Gradhand, Georg Auburger, Suzana Gispert
Published 2020-07-01

Both ubiquitin ligases FBXW8 and PARK2 are sequestrated into insolubility by ATXN2 PolyQ expansions, but only FBXW8 expression is dysregulated.

by Melanie Vanessa Halbach, Tanja Stehning, Ewa Damrath, Marina Jendrach, Nesli Ece Şen, A Nazlı Başak, Georg Auburger
Published 2015-01-01

SerThr-PhosphoProteome of Brain from Aged PINK1-KO+A53T-SNCA Mice Reveals pT1928-MAP1B and pS3781-ANK2 Deficits, as Hub between Autophagy and Synapse Changes

by Georg Auburger, Suzana Gispert, Sylvia Torres-Odio, Marina Jendrach, Nadine Brehm, Júlia Canet-Pons, Jana Key, Nesli-Ece Sen
Published 2019-07-01

Systematic Surveys of Iron Homeostasis Mechanisms Reveal Ferritin Superfamily and Nucleotide Surveillance Regulation to be Modified by PINK1 Absence

by Jana Key, Nesli Ece Sen, Aleksandar Arsović, Stella Krämer, Robert Hülse, Natasha Nadeem Khan, David Meierhofer, Suzana Gispert, Gabriele Koepf, Georg Auburger
Published 2020-10-01

Search for SCA2 blood RNA biomarkers highlights Ataxin-2 as strong modifier of the mitochondrial factor PINK1 levels

by Nesli Ece Sen, Jessica Drost, Suzana Gispert, Sylvia Torres-Odio, Ewa Damrath, Michael Klinkenberg, Hamid Hamzeiy, Gülden Akdal, Halil Güllüoğlu, A. Nazlı Başak, Georg Auburger
Published 2016-12-01

In Human and Mouse Spino-Cerebellar Tissue, Ataxin-2 Expansion Affects Ceramide-Sphingomyelin Metabolism

by Nesli-Ece Sen, Aleksandar Arsovic, David Meierhofer, Susanne Brodesser, Carola Oberschmidt, Júlia Canet-Pons, Zeynep-Ece Kaya, Melanie-Vanessa Halbach, Suzana Gispert, Konrad Sandhoff, Georg Auburger
Published 2019-11-01

Mouse Ataxin-2 Expansion Downregulates CamKII and Other Calcium Signaling Factors, Impairing Granule—Purkinje Neuron Synaptic Strength

by Aleksandar Arsović, Melanie Vanessa Halbach, Júlia Canet-Pons, Dilhan Esen-Sehir, Claudia Döring, Florian Freudenberg, Nicoletta Czechowska, Kay Seidel, Stephan L. Baader, Suzana Gispert, Nesli-Ece Sen, Georg Auburger
Published 2020-09-01

Generation of an Atxn2-CAG100 knock-in mouse reveals N-acetylaspartate production deficit due to early Nat8l dysregulation

by Nesli-Ece Sen, Júlia Canet-Pons, Melanie V. Halbach, Aleksandar Arsovic, Ulrich Pilatus, Woon-Hyung Chae, Zeynep-Ece Kaya, Kay Seidel, Ewa Rollmann, Michel Mittelbronn, David Meierhofer, Chris I. De Zeeuw, Laurens W.J. Bosman, Suzana Gispert, Georg Auburger
Published 2019-12-01

Atxn2-CAG100-KnockIn mouse spinal cord shows progressive TDP43 pathology associated with cholesterol biosynthesis suppression

by Júlia Canet-Pons, Nesli-Ece Sen, Aleksandar Arsović, Luis-Enrique Almaguer-Mederos, Melanie V. Halbach, Jana Key, Claudia Döring, Anja Kerksiek, Gina Picchiarelli, Raphaelle Cassel, Frédérique René, Stéphane Dieterlé, Nina V. Fuchs, Renate König, Luc Dupuis, Dieter Lütjohann, Suzana Gispert, Georg Auburger
Published 2021-05-01