PUBLIKATIONEN
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2023
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Linear ubiquitination induces NEMO phase separation to activate NF-κB signaling
Goel S, Oliva R, Jeganathan S, Bader V, Krause LJ, Kriegler S, Stender ID, Christine CW, Nakamura K, Hoffmann JE, Winter R, Tatzelt J, Winklhofer KF.
Life Sci Alliance 31;6(4): e202201607. -
Loss of parkin causes endoplasmic reticulum calcium dyshomeostasis by upregulation of reticulocalbin 1
Rybarski M, Mrohs D, Osenberg K, Hemmersbach M, Pfeffel K, Steinkamp J, Schmidt D, Violou K, Schäning R, Schmidtke K, Bader V, Andriske M, Bohne P, Mark MD, Winklhofer KF, Lübbert H, Zhu XR.
Eur J. Neurosci. 19; ejn.15917.
2022
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LUBAC assembles a ubiquitin signaling platform at mitochondria for signal amplification and transport of NF-κB to the nucleus
Wu Z, Berlemann LA, Bader V, Sehr DA, Dawin E, Covallero A, Meschede J, Angersbach L, Showkat C, Michaelis JB, Münch C, Rieger B, Namgaladze D, Herrera MG, Fiesel FC, Springer W, Mendes M, Stepien J, Barkovits K, Marcus K, Sickmann A, Dittmar G, Busch KB, Riedel D, Brini M, Tatzelt J, Cali T, Winklhofer KF
EMBO J. 18; e112006. -
ROS scavengers decrease γH2ax spots in motor neuronal nuclei of ALS model mice in vitro
Junghans M, John F, Cihankaya H, Schliebs D, Winklhofer KF, Bader V, Matschke J, Theiss C, Matschke V.
Front Cell Neurosci. 31;16: e963169. -
A ribavirin-induced ORF2 single-nucleotide variant produces defective hepatitis E virus particles with immune decoy function
Meister TL, Brüggemann Y, Nocke MK, Ulrich RG, Schuhenn J, Sutter K, Gömer A, Bader V, Winklhofer KF, Broering R, Verhoye L, Meuleman P, Vondran FWR, Camuzet C, Cocquerel L, Todt D, Steinmann E.
Proc Natl Acad Sci U S A . 23;119(34): e2202653119. -
Brevican, Neurocan, Tenascin-C, and Tenascin-R Act as Important Regulators of the Interplay Between Perineuronal Nets, Synaptic Integrity, Inhibitory Interneurons, and Otx2
Mueller-Buehl C, Reinhard J, Roll L, Bader V, Winklhofer KF, Faissner A.
Front Cell Dev Biol. 2;10: e886527. -
The Role of Ubiquitin in Regulating Stress Granule Dynamics
Krause LJ, Herrera MG, Winklhofer KF
Front Physiol. 25;13: e910759.
2021
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Increased ROS-Dependent Fission of Mitochondria Causes Abnormal Morphology of the Cell Powerhouses in a Murine Model of Amyotrophic Lateral Sclerosis.
Stein J, Walkenfort B, Cihankaya H, Hasenberg M, Bader V, Winklhofer KF, Röderer P, Matschke J, Theiss C, Matschke V.
Oxid Med Cell Longev. 14: 6924251 -
Increased levels of mitochondrial import factor Mia40 prevent the aggregation of polyQ proteins in the cytosol.
Schlagowski AM, Knöringer K, Morlot S, Sánchez Vicente A, Flohr T, Krämer L, Boos F, Khalid N, Ahmed S, Schramm J, Murschall LM, Haberkant P, Stein F, Riemer J, Westermann B, Braun RJ, Winklhofer KF, Charvin G, Herrmann JM.
EMBO J. 40(16): e107913. -
Hypochlorous acid-modified human serum albumin suppresses MHC class II - dependent antigen presentation in pro-inflammatory macrophages.
Ulfig A, Bader V, Varatnitskaya M, Lupilov N, Winklhofer KF, Leichert LI.
Redox Biol. 43: 101981. -
Optogenetic delivery of trophic signals in a genetic model of Parkinson's disease.
Ingles-Prieto A, Furthmann N, Crossman SH, Tichy AM, Hoyer N, Petersen M, Zheden V, Biebl J, Reichhart E, Gyoergy A, Siekhaus DE, Soba P, Winklhofer KF, Janovjak H.
PLoS Genet. 17(4): e1009479. -
Amyloid precursor protein elevates fusion of promyelocytic leukemia nuclear bodies in human hippocampal areas with high plaque load.
Marks D, Heinen N, Bachmann L, Meermeyer S, Werner M, Gallego L, Hemmerich P, Bader V, Winklhofer KF, Schröder E, Knauer SK, Müller T.
Acta Neuropathol Commun. 9(1): 66. -
Remodeling of the Fibrillation Pathway of α-Synuclein by Interaction with Antimicrobial Peptide LL-III
Oliva R, Mukherjee SK, Ostermeier L, Pazurek LA, Kriegler S, Bader V, Prumbaum D, Raunser S, Winklhofer KF, Tatzelt J, Winter RHA.
Chemistry 27(46): 11845-11851. -
The N-terminal domain of the prion protein is required and sufficient for liquid-liquid phase separation: A crucial role of the Aβ-binding domain
Kamps J,Lin YH, Oliva R, Bader V, Winter R, Winklhofer KF, Tatzelt J.
J Biol Chem 297(1): 100860. -
The key role of solvent in condensation: Mapping hydration water in liquid-liquid phase-separated FUS
Ahlers J, Adam EM, Bader V, Pezzotti S, Winklhofer KF, Tatzelt J, Havenith M.
Biophys J, 120(7): 1266-1275.
2020
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Protein quality control by the proteasome and autophagy: A regulatory role of ubiquitin and liquid-liquid phase separation
Lei L, Wu Z, Winklhofer KF.
Matrix Biol. S0945-053X(20): 30105-0 -
SecY-mediated quality control prevents the translocation of non-gated porins.
Jung S, Bader V, Natriashvili V, Koch HG, Winklhofer KF, Tatzelt J.
Sci Rep. 10(1): 16347. -
PINK1 and Parkin: team players in stress-induced mitophagy.
Bader V, Winklhofer KF.
Biol Chem. 401(6-7): 891-899. -
Propionic Acid Shapes the Multiple Sclerosis Disease Course by an Immunomodulatory Mechanism.
Duscha A, Gisevius B, Hirschberg S, Yissachar N, Stangl GI, Eilers E, Bader V, Haase S, Kaisler J, David C, Schneider R, Troisi R, Zent D, Hegelmaier T1, Dokalis N, Gerstein S, Del Mare-Roumani S, Amidror S, Staszewski O, Poschmann G, Stühler K, Hirche F, Balogh A, Kempa S, Träger P, Zaiss MM, Holm JB, Massa MG, Nielsen HB, Faissner A, Lukas C, Gatermann SG, Scholz M, Przuntek H, Prinz M, Forslund SK, Winklhofer KF, Müller DN, Linker RA, Gold R, Haghikia A.
Cell. 180(6): 1067-1080. -
The parkin-coregulated gene product PACRG promotes TNF signaling by stabilizing LUBAC.
Meschede J, Šadić M, Furthmann N, Miedema T, Sehr DA, Müller-Rischart AK, Bader V, Berlemann LA, Pilsl A, Schlierf A, Barkovits K, Kachholz B, Rittinger K, Ikeda F, Marcus K, Schaefer L, Tatzelt J, Winklhofer KF.
Sci Signal. 13(617). -
Linear Ubiquitin Chains: Cellular Functions and Strategies for Detection and Quantification
Dittmar G, Winklhofer KF.
Front Chem. 7: 915. -
Activation leads to a significant shift in the intracellular redox homeostasis of neutrophil-like cells.
Xie K, Varatnitskaya M, Maghnouj A, Bader V, Winklhofer KF, Hahn S, Leichert LI.
Redox Biol. 28: 101344.
2019
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Mitochondria at the interface between neurodegeneration and neuroinflammation.
Bader V, Winklhofer KF.
Semin Cell Dev Biol. (18) 30191-5. -
Synthesis of Indomorphan Pseudo-Natural Product Inhibitors of Glucose Transporters GLUT-1 and -3.
Ceballos J, Schwalfenberg M, Karageorgis G, Reckzeh ES, Sievers S, Ostermann C, Pahl A, Sellstedt M, Nowacki J, Carnero Corrales MA, Wilke J, Laraia L, Tschapalda K, Metz M, Sehr DA, Brand S, Winklhofer K, Janning P, Ziegler S, Waldmann H.
Angew Chem Int Ed Engl. 58(47): 17016-17025. -
A new perspective on membrane-embedded Bax oligomers using DEER and bioresistant orthogonal spin labels.
Teucher M, Zhang H, Bader V, Winklhofer KF, García-Sáez AJ, Rajca A, Bleicken S, Bordignon E.
Sci Rep. 9(1): 13013. -
α-Synuclein in Parkinson's disease: causal or bystander?
Riederer P, Berg D, Casadei N, Cheng F, Classen J, Dresel C, Jost W, Krüger R, Müller T, Reichmann H, Rieß O, Storch A, Strobel S, van Eimeren T, Völker HU, Winkler J, Winklhofer KF, Wüllner U, Zunke F, Monoranu CM.
J Neural Transm (Vienna). 126(7): 815-840. -
A protein quality control pathway regulated by linear ubiquitination.
van Well EM, Bader V, Patra M, Sánchez-Vicente A, Meschede J, Furthmann N, Schnack C, Blusch A, Longworth J, Petrasch-Parwez E, Mori K, Arzberger T, Trümbach D, Angersbach L, Showkat C, Sehr DA, Berlemann LA, Goldmann P, Clement AM, Behl C, Woerner AC, Saft C, Wurst W, Haass C, Ellrichmann G, Gold R, Dittmar G, Hipp MS, Hartl FU, Tatzelt J, Winklhofer KF.
EMBO J. 38(9): doi: 10.15252/embj.2018100730.
News & Views -
Author Correction: Laquinimod treatment in the R6/2 mouse model.
Ellrichmann G, Blusch A, Fatoba O, Brunner J, Reick C, Hayardeny L, Hayden M, Sehr D, Winklhofer KF, Saft C, Gold R.
Sci Rep. 9(1): 4960.
2018
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Alterations in the brain interactome of the intrinsically disordered N-terminal domain of the cellular prion protein (PrPC) in Alzheimer's disease
Ulbrich S, Janning P, Seidel R, Matschke J, Gonsberg A, Jung S, Glatzel M, Engelhard M, Winklhofer KF, Tatzelt J
PLoS One. 13(5): e0197659. -
Dimerization of the cellular prion protein inhibits propagation of scrapie prions
Engelke AD, Gonsberg A, Thapa S, Jung S, Ulbrich S, Seidel R, Basu S, Multhaup G, Baier M, Engelhard M, Schätzl HM, Winklhofer KF, Tatzelt J
J Biol Chem. 293(21): 8020-8031. -
Increased ROS Level in Spinal Cord of Wobbler Mice due to Nmnat2 Downregulation
Röderer P, Klatt L, John F, Theis V, Winklhofer KF, Theiss C, Matschke V
Mol Neurobiol. 55(11): 8414-8424. -
Neutrophil-generated HOCl leads to non-specific thiol oxidation in phagocytized bacteria
Degrossoli A, Müller A, Xie K, Schneider JF, Bader V, Winklhofer KF, Meyer AJ, Leichert LI
Elife. 7 e32288.
2017
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The Sec61/SecY complex is inherently deficient in translocating intrinsically disordered proteins.
Gonsberg A, Jung S, Ulbrich S, Origi A, Ziska A, Baier M, Koch HG, Zimmermann R, Winklhofer KF, Tatzelt J.
J Biol Chem. 292(52): 21383-21396. -
Laquinimod treatment in the R6/2 mouse model.
Ellrichmann G, Blusch A, Fatoba O, Brunner J, Hayardeny L, Hayden M, Sehr D, Winklhofer KF, Saft C, Gold R.
Sci Rep. 7(1): 4947. -
Alpha-synuclein prevents the formation of spherical mitochondria and apoptosis under oxidative stress.
Menges S, Minakaki G, Schaefer PM, Meixner H, Prots I, Schlötzer-Schrehardt U, Friedland K, Winner B, Outeiro TF, Winklhofer KF, von Arnim CA, Xiang W, Winkler J, Klucken J.
Sci Rep. 7: 42942. doi: 10.1038/srep42942. -
PERK activation mitigates tau pathology in vitro and in vivo.
Bruch J, Xu H, Rösler TW, De Andrade A, Kuhn PH, Lichtenthaler SF, Arzberger T, Winklhofer KF, Müller U, Höglinger GU.
EMBO Mol Med. 9(3): 371-384.
2016
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The mitochondrial kinase PINK1: functions beyond mitophagy.
Voigt A, Berlemann LA, Winklhofer KF.
J Neurochem. 139 Suppl 1: 232-239. -
Guidelines for the use and interpretation of assays for monitoring autophagy (3rd edition).
Klionsky et al.
Autophagy 12(1): 1-222. -
Cytoplasmic protein aggregates interfere with nucleo-cytoplasmic transport of protein and RNA.
Woerner AC, Frottin F, Hornburg D, Feng LR, Meissner F, Patra M, Tatzelt J, Mann M, Winklhofer KF, Hartl FU, Hipp MS.
Science 351(6269): 173-176.
2015
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Parkin cooperates with GDNF/RET signaling to prevent dopaminergic neuron degeneration.
Meka DP, Müller-Rischart AK, Nidadavolu P, Mohammadi B, Motori E, Ponna SK, Aboutalebi H, Bassal M, Annamneedi A, Finckh B, Miesbauer M, Rotermund N, Lohr C, Tatzelt J, Winklhofer KF, Kramer ER.
J Clin Invest. 125(5): 1873-1885.
2014
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Parkin - eine neuroprotektive E3-Ubiquitin-Ligase
Schnack C, Berlemann L, Winklhofer KF.
BIOspektrum 20(7): 724-728. -
Parkin and mitochondrial quality control: Towards assembling the puzzle.
Winklhofer KF.
Trends Cell Biol. 24(6): 332-341. -
Ret rescues mitochondrial morphology and muscle degeneration of Drosophila Pink1 mutants.
Klein P, Müller-Rischart AK, Motori E, Schönbauer C, Schnorrer F, Winklhofer KF, Klein R.
EMBO J. 33(4): 341-355.
2013
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Inflammation-Induced Alteration of Astrocyte Mitochondrial Dynamics Requires Autophagy for Mitochondrial Network Maintenance.
Motori E, Puyal J, Toni T, Ghanem T, Angeloni C, Malaguti M, Cantelli-Forti C, Berninger B, Conzelmann K, Götz M, Winklhofer KF, Hrelia S, Bergami M.
Cell Metab. (18)6: 844-859. -
Impaired complex IV activity in response to loss of LRPPRC function can be compensated by mitochondrial hyperfusion.
Rolland SG, Motori E, Memar N, Hench J, Frank S, Winklhofer KF, Conradt B.
Proc Natl Acad Sci U S A. 110(32): 2967-2976. -
The α-helical structure of prodomains promotes translocation of intrinsically disordered neuropeptide hormones into the endoplasmic reticulum.
Dirndorfer D, Seidel RP, Nimrod G, Miesbauer M, Ben-Tal N, Engelhard M, Zimmermann R, Winklhofer KF, Tatzelt J.
J Biol Chem. 288(20): 13961-13973. -
TRAP1 rescues PINK1 loss-of-function phenotypes.
Zhang L, Karsten P, Hamm S, Pogson JH, Müller-Rischart AK, Exner N, Haass C, Whitworth AJ, Winklhofer KF, Schulz JB, Voigt A.
Hum Mol Genet. 22(14): 2829-2841. -
Structural features within the nascent chain regulate alternative targeting of secretory proteins to mitochondria.
Pfeiffer NV, Dirndorfer D, Lang S, Resenberger UK, Restelli LM, Hemion C, Miesbauer M, Frank S, Neutzner A, Zimmermann R, Winklhofer KF, Tatzelt J.
EMBO J. 32(7): 1036-1051. -
The E3 ligase parkin maintains mitochondrial integrity by increasing linear ubiquitination of NEMO.
Müller-Rischart AK, Pilsl A, Beaudette P, Patra M, Hadian K, Funke M, Peis R, Deinlein A, Schweimer C, Kuhn PH, Lichtenthaler SF, Motori E, Hrelia S, Wurst W, Trümbach D, Langer T, Krappmann D, Dittmar G, Tatzelt J, Winklhofer KF.
Mol Cell. 49(5): 908-921.
2012
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The heat shock response is modulated by and interferes with toxic effects of scrapie prion protein and amyloid β.
Resenberger UK, Müller V, Munter LM, Baier M, Multhaup G, Wilson MR, Winklhofer KF, Tatzelt J.
J Biol Chem. 287(52): 43765-43776. -
Mitochondrial dysfunction in Parkinson's disease: molecular mechanisms and pathophysiological consequences.
Exner N, Lutz AK, Haass C, Winklhofer KF.
EMBO J. 31(14): 3038-3062. -
The mitochondrial chaperone protein TRAP1 mitigates α-Synuclein toxicity.
Butler EK, Voigt A, Lutz AK, Toegel JP, Gerhardt E, Karsten P, Falkenburger B, Reinartz A, Winklhofer KF, Schulz JB.
PLoS Genet. 8(2): e1002488. -
Pink1-deficiency in mice impairs gait, olfaction and serotonergic innervation of the olfactory bulb.
Glasl L, Kloos K, Giesert F, Roethig A, Di Benedetto B, Kühn R, Zhang J, Hafen U, Zerle J, Hofmann A, de Angelis MH, Winklhofer KF, Hölter SM, Vogt Weisenhorn DM, Wurst W.
Exp Neurol. 235(1): 214-227. -
Cellular prion protein mediates toxic signaling of amyloid beta.
Resenberger UK, Winklhofer KF, Tatzelt J.
Neurodegener Dis. 10(1-4): 298-300. -
Parkin, PINK1 and mitochondrial integrity: emerging concepts of mitochondrial dysfunction in Parkinson's disease.
Pilsl A, Winklhofer KF.
Acta Neuropathol. 123(2): 173-188.
2011
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Neuroprotective and neurotoxic signaling by the prion protein.
Resenberger UK, Winklhofer KF, Tatzelt J.
Top Curr Chem. 305: 101-119. -
The cellular prion protein mediates neurotoxic signalling of β-sheet-rich conformers independent of prion replication.
Resenberger UK, Harmeier A, Woerner AC, Goodman JL, Müller V, Krishnan R, Vabulas RM, Kretzschmar HA, Lindquist S, Hartl FU, Multhaup G, Winklhofer KF, Tatzelt J.
EMBO J. 30(10) 2057-2070 -
Conserved stress-protective activity between prion protein and Shadoo.
Sakthivelu V, Seidel RP, Winklhofer KF, Tatzelt J.
J Biol Chem. 286(11): 8901-8908. -
Parkin is transcriptionally regulated by ATF4: evidence for an interconnection between mitochondrial stress and ER stress.
Bouman L, Schlierf A, Lutz AK, Shan J, Deinlein A, Kast J, Galehdar Z, Palmisano V, Patenge N, Berg D, Gasser T, Augustin R, Trümbach D, Irrcher I, Park DS, Wurst W, Kilberg MS, Tatzelt J, Winklhofer KF.
Cell Death Differ. 18(5): 769-782.
2010
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Protein immobilization on liposomes and lipid-coated nanoparticles by protein trans-splicing.
Chu NK, Olschewski D, Seidel R, Winklhofer KF, Tatzelt J, Engelhard M, Becker CF.
J Pept Sci. 16(10): 582-588. -
Inhibition of mitochondrial fusion by α-synuclein is rescued by PINK1, Parkin and DJ-1.
Kamp F, Exner N, Lutz AK, Wender N, Hegermann J, Brunner B, Nuscher B, Bartels T, Giese A, Beyer K, Eimer S, Winklhofer KF, Haass C.
EMBO J. 29(20): 3571-3589. -
Parkin is protective against proteotoxic stress in a transgenic zebrafish model.
Fett ME, Pilsl A, Paquet D, van Bebber F, Haass C, Tatzelt J, Schmid B, Winklhofer KF.
PLoS One. 5(7): e11783. -
Loss of the Parkinson's disease-linked gene DJ-1 perturbs mitochondrial dynamics.
Irrcher I, Aleyasin H, Seifert EL, Hewitt SJ, Chhabra S, Phillips M, Lutz AK, Rousseaux MW, Bevilacqua L, Jahani-Asl A, Callaghan S, MacLaurin JG, Winklhofer KF, Rizzu P, Rippstein P, Kim RH, Chen CX, Fon EA, Slack RS, Harper ME, McBride HM, Mak TW, Park DS.
Hum Mol Genet. 19(19): 3734-3746. -
Synthesis of a GPI anchor module suitable for protein post-translational modification.
Schumacher MC, Resenberger U, Seidel RP, Becker CF, Winklhofer KF, Oesterhelt D, Tatzelt J, Engelhard M.
Biopolymers. 94(4): 457-464. -
Targeting of the prion protein to the cytosol: mechanisms and consequences.
Miesbauer M, Rambold AS, Winklhofer KF, Tatzelt J.
Curr Issues Mol Biol. 12(2): 109-118. -
Mitochondrial dysfunction in Parkinson's disease.
Winklhofer KF, Haass C.
Biochim Biophys Acta. 1802(1): 29-44.
2009
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alpha-Helical domains promote translocation of intrinsically disordered polypeptides into the endoplasmic reticulum.
Miesbauer M, Pfeiffer NV, Rambold AS, Müller V, Kiachopoulos S, Winklhofer KF, Tatzelt J.
J Biol Chem. 284(36): 24384-24393. -
Loss of parkin or PINK1 function increases Drp1-dependent mitochondrial fragmentation.
Lutz AK, Exner N, Fett ME, Schlehe JS, Kloos K, Lämmermann K, Brunner B, Kurz-Drexler A, Vogel F, Reichert AS, Bouman L, Vogt-Weisenhorn D, Wurst W, Tatzelt J, Haass C, Winklhofer KF.
J Biol Chem. 284(34): 22938-22951.
2008
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Green tea extracts interfere with the stress-protective activity of PrP and the formation of PrP.
Rambold AS, Miesbauer M, Olschewski D, Seidel R, Riemer C, Smale L, Brumm L, Levy M, Gazit E, Oesterhelt D, Baier M, Becker CF, Engelhard M, Winklhofer KF, Tatzelt J.
J Neurochem. 107(1): 218-229. -
Stress-protective signalling of prion protein is corrupted by scrapie prions.
Rambold AS, Müller V, Ron U, Ben-Tal N, Winklhofer KF, Tatzelt J.
EMBO J. 27(14): 1974-1984. -
Aberrant folding of pathogenic Parkin mutants: aggregation versus degradation.
Schlehe JS, Lutz AK, Pilsl A, Lämmermann K, Grgur K, Henn IH, Tatzelt J, Winklhofer KF.
J Biol Chem. 283(20): 13771-13779. -
The two faces of protein misfolding: gain- and loss-of-function in neurodegenerative diseases.
Winklhofer KF, Tatzelt J, Haass C.
EMBO J. 27(2): 336-349. -
Observing fibrillar assemblies on scrapie-infected cells.
Wegmann S, Miesbauer M, Winklhofer KF, Tatzelt J, Muller DJ.
Pflugers Arch. 456(1): 83-93.
2007
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The parkin protein as a therapeutic target in Parkinson's disease.
Winklhofer KF.
Expert Opin Ther Targets. 11(12): 1543-1552. -
Loss-of-function of human PINK1 results in mitochondrial pathology and can be rescued by parkin.
Exner N, Treske B, Paquet D, Holmström K, Schiesling C, Gispert S, Carballo-Carbajal I, Berg D, Hoepken HH, Gasser T, Krüger R, Winklhofer KF, Vogel F, Reichert AS, Auburger G, Kahle PJ, Schmid B, Haass C.
J Neurosci. 27(45): 12413-12418. -
Semisynthetic murine prion protein equipped with a GPI anchor mimic incorporates into cellular membranes.
Olschewski D, Seidel R, Miesbauer M, Rambold AS, Oesterhelt D, Winklhofer KF, Tatzelt J, Engelhard M, Becker CF.
Chem Biol. 14(9): 994-1006. -
Parkin mediates neuroprotection through activation of IkappaB kinase/nuclear factor-kappaB signaling.
Henn IH, Bouman L, Schlehe JS, Schlierf A, Schramm JE, Wegener E, Nakaso K, Culmsee C, Berninger B, Krappmann D, Tatzelt J, Winklhofer KF.
J Neurosci. 27(8): 1868-1878.
2006
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Association of Bcl-2 with misfolded prion protein is linked to the toxic potential of cytosolic PrP.
Rambold AS, Miesbauer M, Rapaport D, Bartke T, Baier M, Winklhofer KF, Tatzelt J.
Mol Biol Cell. 17(8): 3356-3368. -
The role of chaperones in Parkinson's disease and prion diseases.
Winklhofer KF, Tatzelt J.
Handb Exp Pharmacol. 172: 221-258. -
Structural instability of the prion protein upon M205S/R mutations revealed by molecular dynamics simulations.
Hirschberger T, Stork M, Schropp B, Winklhofer KF, Tatzelt J, Tavan P.
Biophys J. 90(11): 3908-3918. -
Prion protein-related proteins from zebrafish are complex glycosylated and contain a glycosylphosphatidylinositol anchor.
Miesbauer M, Bamme T, Riemer C, Oidtmann B, Winklhofer KF, Baier M, Tatzelt J.
Biochem Biophys Res Commun. 341(1): 218-224.
2005
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The polysaccharide scaffold of PrP 27-30 is a common compound of natural prions and consists of alpha-linked polyglucose.
Dumpitak C, Beekes M, Weinmann N, Metzger S, Winklhofer KF, Tatzelt J, Riesner D.
Biol Chem. 386(11): 1149-1155. -
Systematic identification of antiprion drugs by high-throughput screening based on scanning for intensely fluorescent targets.
Bertsch U, Winklhofer KF, Hirschberger T, Bieschke J, Weber P, Hartl FU, Tavan P, Tatzelt J, Kretzschmar HA, Giese A.
J Virol. 79(12): 7785-7791. -
A pathogenic PrP mutation and doppel interfere with polarized sorting of the prion protein.
Uelhoff A, Tatzelt J, Aguzzi A, Winklhofer KF, Haass C.
J Biol Chem. 280(7): 5137-5140. -
Pathogenic mutations inactivate parkin by distinct mechanisms.
Henn IH, Gostner JM, Lackner P, Tatzelt J, Winklhofer KF.
J Neurochem. 92(1): 114-122. -
Pathogenic mutations located in the hydrophobic core of the prion protein interfere with folding and attachment of the glycosylphosphatidylinositol anchor.
Kiachopoulos S, Bracher A, Winklhofer KF, Tatzelt J.
J Biol Chem. 280(10): 9320-9329.
2004
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Folding and misfolding of the prion protein in the secretory pathway.
Tatzelt J, Winklhofer KF.
Amyloid. 11(3): 162-172. -
Misfolding of the prion protein at the plasma membrane induces endocytosis, intracellular retention and degradation.
Kiachopoulos S, Heske J, Tatzelt J, Winklhofer KF.
Traffic. 5(6): 426-436. -
The C-terminal globular domain of the prion protein is necessary and sufficient for import into the endoplasmic reticulum.
Heske J, Heller U, Winklhofer KF, Tatzelt J.
J Biol Chem. 279(7): 5435-5443.
2003
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Inactivation of parkin by oxidative stress and C-terminal truncations: a protective role of molecular chaperones.
Winklhofer KF, Henn IH, Kay-Jackson PC, Heller U, Tatzelt J.
J Biol Chem. 278(47): 47199-47208. -
Cofactor Tpr2 combines two TPR domains and a J domain to regulate the Hsp70/Hsp90 chaperone system.
Brychzy A, Rein T, Winklhofer KF, Hartl FU, Young JC, Obermann WM.
EMBO J. 22(14): 3613-3623. -
Post-translational import of the prion protein into the endoplasmic reticulum interferes with cell viability: a critical role for the putative transmembrane domain.
Heller U, Winklhofer KF, Heske J, Reintjes A, Tatzelt J.
J Biol Chem. 278(38): 36139-36147. -
Inhibition of complex glycosylation increases the formation of PrPsc.
Winklhofer KF, Heller U, Reintjes A, Tatzelt J.
Traffic. 4(5): 313-322. -
Determinants of the in vivo folding of the prion protein. A bipartite function of helix 1 in folding and aggregation.
Winklhofer KF, Heske J, Heller U, Reintjes A, Muranyi W, Moarefi I, Tatzelt J.
J Biol Chem. 278(17): 14961-14970.
2001
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Geldanamycin restores a defective heat shock response in vivo.
Winklhofer KF, Reintjes A, Hoener MC, Voellmy R, Tatzelt J.
J Biol Chem. 276(48): 45160-45167. -
A sensitive filter retention assay for the detection of PrP(Sc) and the screening of anti-prion compounds.
Winklhofer KF, Hartl FU, Tatzelt J.
FEBS Lett. 503(1): 41-45. -
Intracellular re-routing of prion protein prevents propagation of PrP(Sc) and delays onset of prion disease.
Gilch S, Winklhofer KF, Groschup MH, Nunziante M, Lucassen R, Spielhaupter C, Muranyi W, Riesner D, Tatzelt J, Schätzl HM.
EMBO J. 20(15): 3957-3966.
2000
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Human cytomegalovirus immediate-early gene 2 expression leads to JCV replication in nonpermissive cells via transcriptional activation of JCV T antigen.
Winklhofer KF, Albrecht I, Wegner M, Heilbronn R.
Virology. 275(2): 323-334. -
Cationic lipopolyamines induce degradation of PrPSc in scrapie-infected mouse neuroblastoma cells.
Winklhofer KF, Tatzelt J.
Biol Chem. 381(5-6): 463-469.